Hemolytic crisis symptoms. Diagnosis and treatment of emergency conditions in the clinic of internal medicine Hematological crisis in patients with SLE emergency care

Paroxysmal nocturnal hemoglobinuria, also known as Strübing-Marchiafava disease, Marchiafava-Micheli disease, is a rare disease, a progressive blood pathology that threatens the patient’s life. It is one of the types of acquired hemolytic anemia caused by disturbances in the structure of erythrocyte membranes. Defective cells are subject to premature decay (hemolysis) that occurs inside the blood vessels. The disease is genetic in nature, but is not considered inherited.

The incidence is 2 cases per 1 million people. The incidence is 1.3 cases per million people per year. It predominantly manifests itself in people aged 25-45 years; no dependence of incidence on gender and race has been identified. There are isolated cases of the disease in children and adolescents.

Important: the average age at which the disease is diagnosed is 35 years.

Causes of the disease

The causes and risk factors for developing the disease are unknown. It has been established that the pathology is caused by a mutation in the PIG-A gene, located in the short arm of the X chromosome. The mutagenic factor has not yet been identified. In 30% of cases of nocturnal paroxysmal hemoglobinuria, there is a connection with another blood disease - aplastic anemia.

The formation, development and maturation of blood cells (hematopoiesis) occurs in the red bone marrow. All specialized blood cells are formed from so-called stem, unspecialized cells that have retained the ability to divide. Formed as a result of successive divisions and transformations, mature blood cells enter the bloodstream.

A mutation in the PIG-A gene even in a single cell leads to the development of PNH. Damage to the gene also changes the activity of cells in the processes of maintaining bone marrow volume; mutant cells multiply more actively than normal ones. In the hematopoietic tissue, a population of cells producing defective blood cells is quickly formed. In this case, the mutant clone is not a malignant tumor and can spontaneously disappear. The most active replacement of normal bone marrow cells with mutant ones occurs in the processes of restoration of bone marrow tissue after significant damage caused, in particular, by aplastic anemia.

Damage to the PIG-A gene leads to disruptions in the synthesis of signaling proteins that protect body cells from the effects of the complement system. The complement system is specific blood plasma proteins that provide general immune protection. These proteins bind to damaged red blood cells and melt them, and the released hemoglobin mixes with the blood plasma.

Classification

Based on the available data on the causes and characteristics of pathological changes, several forms of paroxysmal nocturnal hemoglobinuria are distinguished:

Subclinical.
Classic.
Associated with hematopoiesis disorders.

The subclinical form of the disease is often preceded by aplastic anemia. There are no clinical manifestations of the pathology, but the presence of a small number of defective blood cells is detected only during laboratory tests.

On a note. There is an opinion that PNH is a more complex disease, the first stage of which is aplastic anemia.

The classic form occurs with typical symptoms; populations of defective red blood cells, platelets and some types of leukocytes are present in the patient’s blood. Laboratory research methods confirm intravascular destruction of pathologically altered cells; hematopoiesis disorders are not detected.

After suffering from diseases leading to hematopoietic insufficiency, a third form of pathology develops. A pronounced clinical picture and intravascular lysis of red blood cells develop against the background of bone marrow lesions.

There is an alternative classification, according to which there are:

Actually PNH, idiopathic.
Developing as a concomitant syndrome with other pathologies.
Developing as a consequence of bone marrow hypoplasia.

The severity of the disease in different cases is not always related to the number of defective red blood cells. Both subclinical cases with a content of modified cells approaching 90%, and extremely severe cases with replacement of 10% of the normal population, have been described.

Development of the disease

It is currently known that in the blood of patients with paroxysmal nocturnal hemoglobinuria, three types of erythrocytes with different sensitivities to destruction by the complement system may be present. In addition to normal cells, red blood cells circulate in the bloodstream, the sensitivity of which is several times higher than normal. In the blood of patients diagnosed with Marchiafava-Micheli disease, cells were found whose sensitivity to complement was 3-5 and 15-25 times higher than normal.

Pathological changes also affect other blood cells, namely platelets and granulocytes. At the height of the disease, patients experience pancytopenia - an insufficient number of blood cells of different types.

The severity of the disease depends on the ratio between the populations of healthy and defective blood cells. The maximum content of red blood cells that are hypersensitive to complement-dependent hemolysis is achieved within 2-3 years from the moment of mutation. At this time, the first typical symptoms of the disease appear.

The pathology usually develops gradually; acute crisis onset is rare. Exacerbations occur against the background of menstruation, severe stress, acute viral diseases, surgery, treatment with certain drugs (in particular, iron-containing ones). Sometimes the disease worsens when eating certain foods or for no obvious reason.

There is evidence of manifestations of Marchiafava-Micheli disease due to radiation exposure.

The dissolution of blood cells to varying degrees in patients with established paroxysmal nocturnal hemoglobinuria occurs constantly. Periods of moderate progression are interspersed with hemolytic crises, massive destruction of red blood cells, which leads to a sharp deterioration in the patient’s condition.

Outside of a crisis, patients are concerned about manifestations of moderate general hypoxia, such as shortness of breath, attacks of arrhythmia, general weakness, and worsening exercise tolerance. During a crisis, abdominal pain appears, localized mainly in the navel area and in the lower back. Urine turns black, the darkest portion is in the morning. The reasons for this phenomenon have not yet been definitively established. With PNH, a slight pastiness of the face develops, and yellowness of the skin and sclera is noticeable.

On a note! A typical symptom of the disease is stained urine. In about half of known cases, the disease does not manifest itself.
In the periods between crises, patients may experience:
anemia;
tendency to thrombosis;
liver enlargement;
manifestations of myocardial dystrophy;
tendency to inflammation of infectious origin.

When blood cells are destroyed, substances that increase clotting are released, which causes thrombosis. Blood clots may form in the vessels of the liver and kidneys; coronary and cerebral vessels are also susceptible to damage, which can lead to death. Thrombosis localized in the liver vessels leads to an increase in the size of the organ. Disturbances in intrahepatic blood flow lead to degenerative changes in tissue. When the portal vein system or splenic veins are blocked, splenomegaly develops. Disorders of nitrogen metabolism are accompanied by dysfunction of smooth muscles; some patients complain of difficulty swallowing, spasms of the esophagus, and erectile dysfunction is possible in men.
Important! Thrombotic complications in PNH predominantly affect the veins; arterial thrombosis rarely develops.

Video - Paroxysmal nocturnal hemoglobinuria
Mechanisms of development of complications of PNH
Hemolytic crisis is manifested by the following symptoms:
acute abdominal pain caused by multiple thrombosis of small mesenteric veins;
increased jaundice;
pain in the lumbar region;
lowering blood pressure;
increased body temperature;
staining urine black or dark brown.

In rare cases, a “hemolytic kidney” develops, a specific transient form of renal failure accompanied by acute anuria. Due to impaired excretory function, nitrogen-containing organic compounds accumulate in the blood, which are the end products of protein breakdown, and azotemia develops. After the patient recovers from the crisis, the content of formed elements in the blood is gradually restored, jaundice and manifestations of anemia partially fade away.
The most common course of the disease is crisis, interspersed with periods of stable, satisfactory condition. In some patients, the periods between crises are very short, insufficient to restore blood composition. Such patients develop persistent anemia. There is also a variant of the course with an acute onset and frequent crises. Over time, crises become less frequent. In especially severe cases, death is possible, which is caused by acute renal failure or thrombosis of blood vessels supplying the heart or brain.
Important! No daily patterns in the development of hemolytic crises have been identified.

In rare cases, the disease can have a long-term quiet course; isolated cases of recovery have been described.
Diagnostics
In the early stages of the disease, diagnosis is difficult due to the manifestation of scattered nonspecific symptoms. Diagnosis sometimes requires several months of observation. The classic symptom - specific staining of urine - appears during crises and not in all patients. Reasons to suspect Marchiafava-Miceli disease are:
iron deficiency of unknown etiology;
thrombosis, headaches, attacks of pain in the lower back and abdomen for no apparent reason;
hemolytic anemia of unknown origin;
melting of blood cells, accompanied by pancytopenia;
hemolytic complications associated with transfusion of fresh donor blood.

In the diagnostic process, it is important to establish the fact of chronic intravascular breakdown of red blood cells and identify specific serological signs of PNH.
In a complex of studies, if nocturnal paroxysmal hemoglobinuria is suspected, in addition to general urine and blood tests, the following are carried out:
determination of hemoglobin and haptoglobin content in the blood;
immunophenotyping by flow cytometry to identify defective cell populations;
serological tests, in particular the Coombs test.

Differential diagnosis with hemoglobinuria and anemia of other etiologies is necessary; in particular, autoimmune hemolytic anemia should be excluded. Common symptoms are anemia, jaundice, and increased bilirubin in the blood. Enlargement of the liver and/or spleen is not observed in all patients
Signs Autoimmune hemolytic
anemia PNG

Coombs test + -
Increased content of free
hemoglobin in blood plasma - +
Hartmann test (sucrose) - +
Hem's test (acidic) - +
Hemosiderin in urine - +
Thrombosis ± +
Hepatomegaly ± ±
Splenomegaly ± ±
The results of the Hartmann and Hem test are specific for PNH and are the most important diagnostic signs.
Treatment
Relief of a hemolytic crisis is carried out by repeated transfusions of red blood cells, thawed or previously washed many times. It is believed that at least 5 transfusions are needed to achieve a lasting result, however, the number of transfusions may differ from the average and is determined by the severity of the patient’s condition.
Attention! Blood cannot be transfused to such patients without prior preparation. Transfusion of donor blood aggravates the crisis.

For symptomatic elimination of hemolysis, patients can be prescribed Nerobol, but relapses are possible after discontinuation of the drug.
Additionally, folic acid, iron, and hepatoprotectors are prescribed. When thrombosis develops, direct-acting anticoagulants and heparin are used.
In extremely rare cases, the patient is indicated for splenectomy - removal of the spleen.
All of these measures are supportive; they alleviate the patient’s condition, but do not eliminate the population of mutant cells.
The prognosis of the disease is considered unfavorable; the patient's life expectancy after detection of the disease with constant maintenance therapy is approximately 5 years. The only effective treatment is red bone marrow transplantation, which replaces the mutant cell population.
Due to the uncertainty of the causes and risk factors for the development of pathology, prevention as such is impossible.

Acute hemolysis is a severe pathological condition characterized by massive destruction of red blood cells, the rapid occurrence of normochromic hyperregenerative anemia, jaundice syndromes, hypercoagulation, resulting in severe hypoxic, intoxication syndromes, thrombosis, acute renal failure, which pose a threat to the patient’s life.

Treatment of hemolytic crisis in enzymatic erythropathy
(symptomatic taking into account etiopathogenesis):

Prednisolone - 2-3 mg/kg/day - first intravenously, then orally until the reticulocyte count normalizes

Transfusion of washed red blood cells with a hemoglobin content below 4.0 mmol/l (6.5 g/%), (transfusion of red blood cells without selecting an individual donor is dangerous)

Prevention of hypothermia in the presence of cold autoAT

Splenectomy in chronic cases (if corticosteroid therapy is ineffective for 6 months)

Principles of emergency treatment

1. Elimination of the action of the etiological factor

2. Detoxification, disaggregation, anti-shock measures, fight against acute renal failure

3. Suppression of antibody formation (during immune genesis).

4. Replacement blood transfusion therapy.

5. Gravity surgery methods

First aid

Rest, warming the patient, hot sweet drink

For cardiovascular failure - dopamine, adrenaline, oxygen inhalation

For severe pain, IV analgesics.

In case of autoimmune HA, transfusions of blood incompatible with blood group and Rh factor, it is advisable to administer drugs

In case of immune genesis of hemolysis (including post-transfusion) - prednisolone 90-200 mg IV bolus

Qualified
and specialized medical care

Detoxification therapy: rheopolyglucin, 5% glucose, saline solution including solutions of acesol, disol, trisol up to 1 l/day intravenously in a heated drip (up to 35°); sodium bicarbonate 4% 150 - 200.0 ml intravenous drip; enterodesis orally 5 g in 100 ml boiled water 3 times a day

Maintain diuresis of at least 100 ml/h with intravenous fluid administration and diuretics

Excretion of free hemoglobin can be increased by alkalinizing the urine. To do this, sodium bicarbonate is added to IV fluids, which increases urine pH to > 7.5

Correction of microcirculation and hemorheology disorders: heparin 10-20 thousand units/day, rheopolyglucin 200-400.0 ml IV drip, Trental 5 ml IV drip in 5% glucose, chimes 2 ml IM

Antihypoxants - sodium hydroxybutyrate 20% 10 -20 ml intravenous drip

Antioxidants (especially during a crisis of paroxysmal nocturnal hemoglobinuria, hemolytic disease of newborns) - tocopherol acetate 5, 10, 30% solution in oil, 1 ml IM (warm to body temperature), aevit 1.0 ml IM or orally 0 .2 ml 2-3 times a day

Prevention and treatment of hemosiderosis - desferal IM or IV drip 500-1000 mg/day

Administration of heparin for the prevention of hemolytic-uremic syndrome in hemolytic anemia caused by neuraminidase, as well as transfusion of washed red blood cells (free from anti-T-Ag)

In severe condition, a decrease in hemoglobin less than 80 g/l and Er less than 3X1012 g/l - transfusions of washed (1, 3, 5, 7 times) red blood cells or red blood mass with selection using the Coombs test

For acute immune hemolysis - prednisolone 120-60-30 mg/day - according to a decreasing regimen

Cytostatics - azathioprine (125 mg/day) or cyclophosphamide (100 mg/day) in combination with prednisone when other therapy does not help. Sometimes - vincristine or the androgenic drug danazol

Immunoglobulin G 0.5-1.0 g/kg/day IV for 5 days

Plasmapheresis, hemosorption (removal of immune complexes, microclots, toxins, pathological metabolites)

Splenectomy for microspherocytosis, chronic autoimmune GA, a number of enzymopathies

Treatment of DIC syndrome, acute renal failure in full

CRISES(French) crisis fracture, attack) is a term used to designate sudden changes in the body, which are characterized by a paroxysmal appearance or intensification of symptoms of the disease and are transient in nature. Systematization of Crises is extremely complex, because this term refers to phenomena that often differ in pathogenesis and wedge, manifestations. Thus, the terms “blast crisis”, “reticulocyte crisis” are used in hematology to designate acute changes in blood composition in leukemia, pernicious anemia; in ophthalmology the terms “glaucomatous crisis” and “glaucomocyclic crisis” are often used for glaucoma; in surgery - “rejection crisis” during organ or tissue transplantation; in neurology - “myasthenic. crisis" with myasthenia gravis, "tabetic crisis" with tabes dorsalis, "solar crisis" with solaritis; in gastroenterology - “stomach, intestinal crisis.” The listed K. refer to the natural manifestations of certain pathols, conditions or diseases. Along with them, there is another group of K., which act as a leading wedge, a sign of the disease. This group includes cerebral K., hypertensive K., thyrotoxic, addisonic, catecholamine, hypercalcemic, hemolytic, erythremic and some others.

Cerebral crises

Cerebral crises can be divided into primary and secondary. Primary cerebral K. develop with functional or organic damage to the brain, Ch. arr. due to a disorder of the centers regulating autonomic functions, including vascular tone, and the functions of a number of internal organs. Thus, in their essence they are more often cerebral vegetative K. However, the wedge, manifestations of primary cerebral K. can be a consequence of dysfunction of other parts of the brain. Depending on the location of the lesion or dysfunction of the brain, blood cells can be temporal, hypothalamic (diencephalic) or brain stem. Secondary cerebral K. (visceral-cerebral K.) are characterized by neurol, disorders caused by somatic diseases.

A special place is occupied by vascular cerebral K., which manifest themselves as unstable disorders of brain function as a result of transient cerebrovascular accident and can be either primary or secondary.

Depending on the volume and localization of vascular changes in the brain, generalized cerebral K. and regional (covering a separate vascular basin) are distinguished.

The pathogenesis of primary cerebral K. is complex. In their origin, disruption of the functions and state of the limbic-reticular system, as well as the endocrine glands, is of great importance. These disorders are manifested by cerebral autonomic crisis, mono- or polysymptomatic. In this case, the reflex mutual regulation between individual functions, which underlies the provision of all homeostatic and adaptive functions of a person, is lost (see Adaptation, Homeostasis).

When the process is localized in the upper parts of the brain stem, in the area of the vestibular nuclei and nuclei of the vagus nerve, which are closely interconnected, there is a predominance of the parasympathetic direction of cerebral K. Similar K. can also occur with damage to the anterior parts of the hypothalamus. Damage to the posterior parts of the hypothalamic region, in which adrenergic structures are most represented, having a special connection with the adaptation apparatus, leads to the development of sympathetic-adrenal K.

Cerebral vascular K. is based on either the mechanism of cerebral vascular insufficiency, or microembolism, or angiodystonic phenomena with a change in the permeability of the vascular wall. Vascular cerebral K., which occurs through the mechanism of cerebrovascular insufficiency, is often caused by the influence of extracerebral factors (changes in blood pressure, a drop in cardiac activity, blood loss, etc.), which, in the presence of stenosis of one of the vessels supplying the brain, cause the development of cerebral ischemia due to a decrease in blood flow into the basin of this vessel. This mechanism occurs especially often in atherosclerosis.

The development of vascular blood vessels can also be facilitated by disturbances in the nervous regulation of cerebral circulation. In cerebral K., cerebral ischemia is usually shallow and short-lived, and therefore focal cerebral symptoms disappear after restoration of cerebral blood flow. Microembolisms that underlie some vascular cerebral K. in atherosclerosis, rheumatism, and vasculitis of various etiologies are cardiogenic (from cardiosclerosis, heart defects, myocardial infarction) and arteriogenic (from the aortic arch and main arteries of the head). The source of emboli are small pieces of parietal thrombi, cholesterol crystals and atheromatous masses from disintegrating atherosclerotic plaques, as well as platelet aggregates. Blockage of a small vessel by an embolus, accompanied by perifocal edema, leads to the appearance of focal symptoms that disappear after the disintegration or lysis of the embolus and reduction of edema or after the establishment of full collateral circulation. In some cases, transient cerebral symptoms that develop without pronounced fluctuations in blood pressure are caused by changes in physical and chemical conditions. properties of blood: an increase in its viscosity, an increase in the number of formed elements, a decrease in oxygen content, hypoglycemia, etc. These factors, in conditions of a decrease in blood supply to the brain, can lead to a drop below the critical level in the amounts of oxygen and glucose delivered to the brain tissue, to a delay in the removal of metabolic end products , especially in the area of the affected vessel, which leads to the appearance of focal symptoms. According to E. V. Schmidt (1963), cerebral vascular K. are often observed against the background of an atherosclerotic stenotic process in the extracranial parts of the vertebral and carotid arteries; sometimes K. arise in patients with patol, tortuosity and kinks of these vessels, as a result of which, in certain positions of the head, disruption of cerebral blood flow may occur. Osteochondrosis of the cervical spine in combination with atherosclerosis of the main arteries of the head often causes the occurrence of regional vascular K., caused by the fact that osteophytes in the area of the uncovertebral joints, during forced turns of the head, compress the vertebral artery passing nearby.

The basis of the pathogenesis of cerebral K. in congenital heart defects are disorders of general hemodynamics, hron, hypoxemia caused by circulatory failure in the systemic circle, anomalies in the development of cerebral vessels. K. in patients with acquired heart defects are caused by a transient insufficiency of blood supply to the brain due to weakening of cardiac activity and fluctuations in blood pressure leading to brain hypoxia. In case of coronary heart disease, cerebral K. arise as a result of patol, afferent impulses, promoting the involvement of peripheral and central parts of the blood in the process. n. With. Various cerebral K., arising from cardiac arrhythmias, are caused by acute cerebral circulatory failure, causing cerebral hypoxia.

Cerebral vascular K. in diseases of the gastrointestinal tract. tract are caused by patol, impulses from the reflexogenic zones of the affected organ to the segmental spinal autonomic centers with the subsequent spread of irritation to the central autonomic formations (limbic-reticular system), which causes secondary cerebral angiodystonic disorders. In the pathogenesis of cerebral K. in liver failure, disturbances of various types of metabolism are important, with intoxication playing a leading role. The basis of the pathogenesis of cerebral K. in acute and chronic renal failure is metabolic disorders, the development of azotemia, acidosis.

Pathomorphol, changes are described only in cerebral vascular K. These data were obtained on the basis of a study of the brain of patients who died during K., complicated by cerebral edema, acute left ventricular or renal failure, or (much less often) acute development of perforated ulcers of the stomach and intestines. Morfol, changes in the brain with cerebral vascular K. may consist in the impregnation of vascular walls with protein masses and blood, accompanied by their focal necrobiosis, sometimes with parietal thrombosis (see), in the development of miliary aneurysms (see), in small perivascular hemorrhages ( see) and plasmorrhagia (see), the appearance of foci of perivascular) melting (encephalolysis), sometimes in focal or diffuse edema (see), focal loss of nerve cells, proliferation of astrocytes (diffuse or focal). Each vascular K., no matter how mild it is, usually leaves behind changes.

Wedge, the picture of cerebral K. is polymorphic. Cerebral K., caused by neuroses (see), occur with a predominance of cardiovascular disorders. With organic damage to the temporal structures (mainly the right hemisphere), cerebral K. are characterized by complex psychopathols, phenomena that include olfactory and auditory hallucinations (see), states of depersonalization (see) and derealization (see). In this case, vegetative-visceral disorders are usually pronounced with a tendency towards a parasympathetic direction.

Hypothalamic K. are very diverse in wedge, manifestations (see Hypothalamic syndrome). Sometimes Hypothalamic K. occurs in the form of Gowers syndrome: attacks of pain in the epigastric region, lasting approx. 30 minutes, accompanied by pallor of the skin, irregular breathing rhythm, cold sweat, fear of death and sometimes ending in vomiting and polyuria. With lesions of the brain stem wedge, the picture of K. is varied, but more often, especially with caudal localization of the process, vagoinsular K. occurs.

Cerebral vascular K. in the domestic literature are usually called transient cerebrovascular accidents (TCI), in foreign literature - transient ischemic attacks. Transient include those cases of cerebral circulation disorders in which the wedge, symptoms persist no longer than 24 hours.

Cerebral vascular K. in atherosclerosis (see) often occurs without cerebral symptoms or the latter are mildly expressed, as well as vegetative ones, but paleness of the face and increased sweating are often observed; Blood pressure in most cases is normal, less often low or moderately high. The most characteristic is the sudden development of transient focal brain symptoms. K.'s development is often provoked by physical and mental overstrain, emotional overload, painful attacks, overheating, neuroendocrine changes that occur during menopause, and sudden meteorolemia. changes.

If cerebral vascular K. is caused by discirculatory disorders in the internal carotid artery, which supplies most of the cerebral hemisphere, then focal symptoms often manifest themselves as paresthesia in the form of numbness, sometimes with a tingling sensation in the skin of the face or limbs on the opposite side; often paresthesias appear simultaneously in half of the upper lip, tongue, on the inner surface of the forearm, and hand. Paralysis or paresis of the muscles of the face and tongue on the opposite side may occur, as well as speech disorders in the form of motor or sensory aphasia (see), apractical disorders, loss of the opposite field of vision (see Hemianopsia), disturbance of the body diagram, etc. Transient crossed optics -pyramidal syndrome (reduced vision or complete blindness in one eye and paresis of the opposite limbs) is considered pathognomonic for stenosis or occlusion of the internal carotid artery in the neck (see Alternating syndromes). Transient visual impairment on the side of the insufficiently functioning carotid artery and paresthesia on the opposite half of the body in hypertension are described as Petzl crises.

Cerebral vascular K., caused by discirculation in the basin of the vertebral and basilar arteries, is characterized by stem symptoms: dizziness of a systemic nature, impaired coordination, swallowing, double vision, nystagmus, dysarthria, bilateral patol, reflexes. Various visual and optical-vestibular disorders, short-term memory loss, and orientation disturbances associated with discirculation in the posterior cerebral artery basin also often appear (see Cerebral circulation).

Wedge, manifestations of vascular cerebral K. in vasculitis, diabetes mellitus and blood diseases are similar to atherosclerotic cerebral K., therefore, one should take into account the features of the somatic disease in which K. occurs.

Wedge, the picture of cerebral vascular K. in hypertension or symptomatic arterial hypertension is characterized by a rapid and significant increase in blood pressure, pronounced cerebral and autonomic symptoms.

Cerebral vascular K. with hypotension develop against the background of low blood pressure and are characterized by pallor of the skin, weakening of the pulse, increased sweating, general weakness, dizziness, and a feeling of blurred vision (see Arterial hypotension).

Viscerocerebral K. often occur with various heart diseases, according to the wedge, their manifestations are polymorphic (see Cardiocerebral syndromes). Thus, with congenital heart defects, cephalgic K., syncope (see Fainting), epileptiform, cyanotic-dyspneic K. are possible. The appearance of attacks of loss of consciousness in patients with “blue” heart defects is a formidable symptom. Cephalgic and syncopal K. also occur in patients with acquired heart defects. In coronary heart disease, cardiocerebral K. are expressed in the appearance of transient focal cerebral symptoms, as well as a variety of vegetative symptoms. Wedge, manifestations of cerebral K. that occur with cardiac arrhythmias include loss of consciousness, cephalalgia, and dizziness. Thus, with Morgagni-Adams-Stokes syndrome, simple or convulsive types of fainting are observed; with paroxysmal tachycardia (see) and atrial fibrillation, fainting, pale face, dizziness and other transient symptoms may occur. A variety of cerebral K. (migraine- and meniere-like, fainting) occur with peptic ulcers of the stomach and duodenum, as well as with diseases of the liver and biliary tract. In patients with hron, pancreatic insufficiency, K. manifests itself in the form of cerebral vascular disorders, hypoglycemic conditions. Various cerebral K. can also be observed in acute and chronic renal failure.

The duration of cerebral vascular K. ranges from several minutes to days. The outcome in most cases is favorable, however, hypertensive cerebral K. can sometimes be followed by cerebral edema or severe left ventricular failure, pulmonary edema and result in death. The course and outcomes of cerebral K. with focal lesions of the brain are usually determined by the nature of the organic process against which K. arises. The course of viscerocerebral K. also depends mainly on the nature and severity of the disease of the internal organs that caused K. Viscerocerebral K. more often arise in period of exacerbation of somatic disease; regression of cerebral disorders also occurs as the function of internal organs improves.

Treatment

Therapy of primary cerebral K. is carried out taking into account the underlying disease, the topic of damage to the nervous system and the initial tone of the autonomic nervous system in the interictal period. If in primary cerebral K. sympathetic tone predominates, adrenolytic substances are used (aminazine, propazine, pyrroxan, ergo- and dihydroergotamine), antispasmodics, vasodilators and hypotensive drugs - reserpine, papaverine, dibazol, nicotinic acid, xanthinol nicotinate (complamin, xavin ), cinnarizine (stugeron). It is also recommended to administer lytic mixtures and, occasionally, ganglion-blocking agents. Increased tone of the parasympathetic department c. n. With. in case of primary cerebral K., it is necessary to prescribe centrally acting anticholinergic drugs internally: cyclodol a (Artane, Parkinsan), amizil a, etc. Calcium preparations are administered intravenously. If these K. are accompanied by allergic symptoms, antihistamines are used (diphenhydramine, pipolfen, suprastin, tavegil). With dysfunction of both departments c. n. With. agents that have adrenergic and anticholinergic effects are used: belloid, bellataminal, bellaspon. In case of severe K., it is necessary to administer cardiovascular drugs (cordiamin, camphor, adrenaline, mesaton).

When treating cerebral vascular K. of atherosclerotic origin, attention should be paid to maintaining blood pressure at a normal level, improving cardiac activity, and using vasodilators. For heart failure, 0.25-1 ml of 0.06% corglycone solution or 0.05% strophanthin solution in 10-20 ml of 20% glucose solution is administered intravenously, as well as cordiamin, camphor oil subcutaneously. In case of a sharp drop in blood pressure, 1% mezaton solution is prescribed subcutaneously (0.3-1 ml) or intravenously (0.1-0.3-0.5 ml 1% solution in 40 ml 5-20-40% solution -ra glucose), caffeine and ephedrine subcutaneously. To improve cerebral blood flow, intravenous or intramuscular administration of aminophylline is prescribed. In some cases, it is possible to use anticoagulants under the control of the blood coagulation system. There is data indicating the prospects of using antiplatelet agents for repeated cerebral vascular K. of atherosclerotic origin - drugs that prevent the formation of platelet aggregates, in particular acetylsalicylic acid, prodectin.

For hypotonic K., caffeine 0.1 g orally, ephedrine 0.025 g orally, mesaton 1 ml of 1% solution or cortin - 1 ml subcutaneously, and sedatives are prescribed.

Viscerocerebral K. require complex treatment, which is carried out depending on the nozol, the form of somatic disease, and also on the nature of the K.

Hypertensive crises

Hypertensive crises are observed in patients suffering from hypertension (see) or arterial hypertension (see Arterial hypertension).

The occurrence of hypertensive blood pressure is characterized by a cyclical nature. Factors contributing to their occurrence may be psycho-emotional stress, hormonal changes in women (menstrual cycle, menopause), meteorol. influences, etc.

The pathogenetic mechanisms of hypertensive K. are not fully disclosed; More often, arterial hypertension occurs in response to emotional stress, accompanied by the formation of foci of excitation in the structures of the c. n. With.

Hypothalamoreticular formations are most closely related to the occurrence of vascular hypertensive reactions. Under normal conditions, the pressor effect is opposed by powerful depressor baroreceptor and humoral influences (prostaglandins, kinins, etc.), acting on the principle of self-regulation.

Hypertensive K. is accompanied by changes in the pituitary-adrenal system, which is manifested by increased secretion of ACTH, vasopressin, glucocorticoids and aldosterone. During K., the content of catecholamines in the blood and their excretion in the urine increases. The effect of these pressor agents on the reactivity and tonic contraction of arterioles is realized to a large extent through their influence on the active transport of ions (an increase in the intracellular content of sodium and calcium).

Excitation of the hypothalamoreticular structures of the brain can lead to disturbances in intrarenal hemodynamics: a persistent decrease in blood flow in the renal cortex and a transient increase in blood flow in the medulla. As a result of ischemia of the renal cortex, the production of renin increases, and the increase in blood flow in the renal medulla promotes increased formation of renal prostaglandins and kinins, which counteract the hypertensive response. The ability of the kidneys to produce humoral substances with pressor and depressor effects depends on the degree and duration of disturbances in intrarenal hemodynamics. Increased production of renin leads to increased formation of angiotensin, which in turn stimulates the production of aldosterone.

The occurrence of hypertensive blood pressure, its severity and consequences are largely determined by the state of the mechanisms of autoregulation of cerebral blood flow. Experimental studies on rabbits have shown that when the reactivity of the subcortical centers changes, the usual adaptive depressor reflex from the baroreceptors of the carotid sinus weakens, changes to a pressor reflex and can cause the occurrence of hypertensive K.

Hypertensive blood pressure is accompanied by a rise in blood pressure. Usually there is a severe headache, often of a bursting nature, pain in the eyeballs - spontaneous and aggravated by eye movement, nausea, sometimes vomiting, noise and ringing in the ears, non-systemic dizziness. Patients experience feelings of anxiety and tension; sometimes there is psychomotor agitation or, on the contrary, drowsiness and stupor. Of the vegetative symptoms, the most common are a feeling of heat in the face, hyperemia or pallor, tachycardia, chills, paresthesia in the limbs and back, polyuria. In severe cases, meningeal symptoms may be observed. Lumbar puncture reveals increased cerebrospinal fluid pressure. Focal neurol symptoms also occur, often mildly expressed; sometimes focal or general epileptic seizures are observed; in the fundus - swelling of the discs (nipples) of the optic nerves, pinpoint hemorrhages.

According to the wedge, course and hemodynamic parameters, two types of hypertensive crises are distinguished. K. of the first type (hyperkinetic) develop rapidly, proceed relatively easily, and are accompanied by severe vegetative-vascular disorders (headache, agitation, tremors, tachycardia). At the moment of K., predominantly systolic and pulse pressure increases; minute blood volume, venous pressure and blood flow velocity increase significantly, but the total peripheral resistance to blood flow does not increase and may even decrease. K. usually ends after 1-3 hours, and sometimes there is profuse urination. Such K. occur Ch. arr. in patients with early stages of hypertension (I or II A).

Hypertensive K. of the second type are much more severe. In the clinic, the leading symptoms are brain symptoms: severe headache, dizziness, drowsiness, nausea and vomiting. Often these K. are accompanied by transient visual disturbances, other focal neurol, symptoms. With such K., not only systolic, but especially diastolic pressure increases. Minute blood volume and venous pressure often do not change, but the total peripheral resistance to blood flow increases significantly. This is the so-called eukinetic variant of hypertensive K. In the presence of coronary heart disease, type 2 K can occur with reduced cardiac output, but a significantly increased general peripheral resistance to blood flow (hypokinetic variant). Crises of the second type usually occur in patients with stage II B and III hypertension, last 3-5 days, and can be complicated by acute coronary insufficiency, left ventricular failure, and focal cerebrovascular accidents. In some cases, during K. an increased amount of patol and elements in the urine sediment is detected.

There are also cardiac hypertensive K., in which in the wedge, the picture is dominated by a disturbance of cardiac activity. According to the wedge, the manifestations distinguish three variants of hypertensive cardiac K. 1) asthmatic, 2) anginal with myocardial infarction, 3) arrhythmic.

In the first option, a sharp increase in blood pressure is accompanied by acute left ventricular failure with attacks of cardiac asthma (see), and in severe cases with pulmonary edema (see). In the second option, against the background of a sharp increase in blood pressure, in addition to cardiac asthma, attacks of angina pectoris and the development of myocardial infarction are observed. The third variant of hypertensive cardiac K. is accompanied by a sudden sharp tachycardia, which may be caused by paroxysmal tachycardia or paroxysm of atrial fibrillation.

Treatment

To relieve hypertensive blood pressure, antihypertensive drugs are used.

In case of hypertensive blood pressure of the first type, the patient's condition allows the use of medications that lower blood pressure 1.5-2 hours after their administration. The drug of choice may be reserpine (rausedil). The drug is administered intramuscularly at a dose of 1.0-2.5 mg. If necessary, the drug is re-administered after 4-6 hours. The total dose per day should not exceed 5 mg. A combination of reserpine with furosemide at a dose of 80 mg orally or ethacrine at a dose of 100 mg orally is more effective. Intramuscular or intravenous administration of 0.5% dibazole solution in a dose of 6-12 ml is also indicated. Magnesium sulfate for the relief of hypertensive K. type 1 is administered intramuscularly or intravenously (slowly) in a dose of 10-20 ml of 25% solution.

Hypertensive K. of the second type require a rapid, within 10-15 minutes, reduction in blood pressure and elimination of hypervolemia and cerebral edema. For this purpose, clonidine (hemiton, catapressan, clonidine) is administered intravenously or intramuscularly at a dose of 0.15-0.30 mg. The effect occurs within 10-15 minutes. If necessary, a second injection is prescribed after 1-4 hours. Clonidine inhibits the release of norepinephrine in the medulla oblongata; its effect is in many ways similar to the effect of ganglion blockers. A rapid and strong decrease in vascular tone in the systemic and pulmonary circulation is achieved by administering ganglion blockers - benzohexonium and pentamine (under blood pressure control). Non thymine is slowly injected into a vein in a dose of 0.2-0.5-0.75 ml of 5% solution, diluted in 20 ml of isotonic sodium chloride solution. For intramuscular injection, use 0.3-0.5-1 ml of 5% pentamine solution. The hypotensive effect of pentamine administered intramuscularly can be enhanced by droperidol (1-3 ml of 0.25% solution intramuscularly). Ganglioblockers are especially indicated during the development of left ventricular heart failure during K. Arfonad (trimetaphan, camsilate) is a ganglion blocker that is used to urgently lower blood pressure in case of intractable arterial hypertension and cerebral edema. The drug is administered intravenously (500 mg of arfonade per 500 ml of 5% glucose solution), starting from 30-50 drops per minute and gradually increasing to 120 drops per minute until the desired effect is obtained.

Diuretics (furosemide, dichlothiazide, hypothiazide) can be of great help in eliminating hypervolemia and cerebral edema. They are prescribed parenterally in combination with the above drugs.

Catecholamine crises

Catecholamine crises are typical for pheochromocytoma (see). They are characterized by a sudden significant rise in blood pressure and a variety of autonomic and metabolic disorders. They are based on hyperproduction of catecholamines (see), in particular adrenaline and norepinephrine. Arterial hypertension is caused not only by the vasoconstrictor effect of catecholamines, but is also associated with activation of the renin-angiotensin-aldosterone system.

Catecholamine K. in pheochromocytoma can be provoked by physical. overstrain, neuro-emotional impact, pressure on the tumor, but often the immediate cause remains unclear. The crisis is developing rapidly. The patient is pale, covered in sweat, very excited, trembling, and experiencing a feeling of fear. He complains of severe headache and dizziness, chest pain. Systolic pressure increases sharply (up to 250-300 mm Hg), diastolic pressure can remain at the same level or also increase (up to 150-170 mm Hg). There is tachycardia and cardiac arrhythmia in the form of extrasystole or atrial fibrillation. Leukocytosis with eosinophilia in the peripheral blood, low glycemia and glycosuria are characteristic. A huge amount of catecholamines is determined in the urine, much more than with hypertensive K. Catecholamine K. lasts from several minutes to several hours and ends suddenly. Sometimes during the period of exit from K. there is a sharp drop in blood pressure, up to collapse.

Treatment

Treatment of catecholamine K. involves the use of adrepolit and ches to their agents, which block the action of catecholamines at the effector level and thereby reduce blood pressure. The most commonly used drugs are phentolamine (Regitine) and tropafene. Phentolamine is administered intramuscularly in 1 ml of 0.5% solution. Tropafen is prescribed intravenously 1 ml of 2% solution.

Thyrotoxic crises

Thyrotoxic crises are a severe complication of thyrotoxicosis (see). A crisis can be triggered by any significant external irritant (stressor), infection, physical. or mental trauma, overheating, strumectomy with insufficient preoperative preparation (so-called postoperative K.). In some cases, the immediate cause of K. remains unclear. The pathogenesis of thyrotoxic K. is caused by the entry into the blood of large quantities of thyroid hormones, leading to sudden changes in the function of the liver, adrenal glands, and heart.

Thyrotoxic K. is characterized by an acute onset and lightning-fast course. Clinically, thyrotoxic K. is manifested by severe mental agitation, often with delirium and hallucinations, sharp tremor of the limbs, sharp tachycardia (up to 150-200 pulses per minute), sometimes with paroxysms of atrial fibrillation, severe sweating, uncontrollable vomiting, diarrhea; fever develops. A large amount of acetone is detected in the urine. Characterized by a decrease in the function of the adrenal cortex, up to acute adrenal insufficiency. Sometimes jaundice appears, which can be combined with acute fatty degeneration of the liver. K.'s duration varies from 2 to 4 days. In severe cases, a coma develops (see Coma) with a fatal outcome. The cause of death may be heart failure, acute fatty liver, or insufficiency of the adrenal cortex.

Treatment

Treatment of thyrotoxic K. consists of eliminating dehydration and intoxication and combating the phenomena of acute insufficiency of the adrenal cortex. 2-3 liters of isotonic sodium chloride solution with 5% glucose solution, 150-300 mg of hydrocortisone or equivalent doses of prednisolone are administered intravenously per day. Sedatives, reserpine, and cardiac glycosides are prescribed. In order to suppress the secretion of thyroid hormones, it is recommended to prescribe thyreostatics (Mercazolil); Sometimes intravenous administration of 1% Lugol's solution, prepared with sodium iodide instead of potassium, is carried out in the amount of 100-250 drops in 1 liter of 5% glucose solution. In K.'s therapy, anaprilin (Inderal) can be used at a dose of 0.04-0.06 g per day. In extremely severe forms, local hypothermia is used.

Hypercalcemic crises

Hypercalcemic crises are most often a complication of primary hyperparathyroidism (see), caused by adenoma or hyperplasia of the parathyroid glands. The main pathogenetic factor is hypercalcemia (see). The development of calcium is associated with calcium intoxication when its concentration in the blood exceeds a critical level (14-17 mg%).

Hypercalcemic K. develops suddenly as a result of the action of some provoking factor: rough palpation of the parathyroid gland area, the prescription of a calcium-rich dairy diet or antacid drugs to a patient with hyperparathyroidism. The initial sign of K. is often abdominal pain localized in the epigastrium. Nausea appears or intensifies, eventually turning into uncontrollable vomiting, accompanied by thirst, and the temperature rises. Severe joint pain, myalgia, muscle weakness, and cramps are noted. The ECG shows sinus tachycardia and shortening of the Q-T interval. Lethargy, confusion, and then coma (against the background of vascular collapse and azotemia) quickly develop. Coma usually occurs with hypercalcemia reaching 20 mg%. K. may result in the death of the patient.

Sometimes hypercalcemic K. is accompanied by acute metastatic pulmonary calcification, acute renal failure, and acute pancreatitis.

Treatment

In case of hypercalcemic K., it is important to create forced diuresis using furosemide, prescribed at a dose of 100 mg/hour with isotonic sodium chloride solution intravenously, and the use of hemodialysis with calcium-free dialysate. Urgent surgery to remove parathyroid adenoma or hyperplastic parathyroid glands is the treatment of choice in cases of primary hyperparathyroidism causing hypercalcemic K.

Hypocalcemic crises

Hypocalcemic crises are a condition opposite to hypercalcemic K., i.e., acute tetany develops (see).

Most often, hypocalcemic K. occurs as a complication of operations on the thyroid gland. Other causes may include idiopathic hypoparathyroidism with insensitivity to parathyroid hormone; damage to the parathyroid glands by a metastatic or infiltrative tumor process: a sharp deficiency in the body of vitamin D or magnesium ions; hypocalcemia with the administration of large doses of calcitonin, glucagon, mithromycin, phosphorus salts, and long-term use of phenobarbital. The main pathogenetic mechanism of hypocalcemic K. is a severe calcium deficiency in the body. K. develops when total calcium decreases to 7.5 mg% and below, and ionized calcium to 4.3 mg% and below.

K. is characterized by muscle spasms, convulsions, difficulty breathing, and on the ECG the Q-T interval is lengthened. During severe K., asphyxia may occur due to spasm of the muscles of the larynx.

Treatment

For hypocalcemic K., intravenous administration of 10-20 ml of 10% gluconate or calcium chloride is indicated.

Addisonian crises

Addisonian crises develop in patients with chronic, adrenal insufficiency (see Addison's disease) with inadequate treatment, the addition of intercurrent infections and intoxications, as well as as a result of surgery for concomitant diseases. The mechanism of occurrence of K. in Addison's disease is due to a rapid and sharp increase in the deficiency of mineralo- and glucocorticoids.

K. develops, as a rule, acutely within several hours. The onset of K. is manifested by a rapid increase in the symptoms of Addison's disease. The general condition sharply worsens, general weakness increases, appetite sharply decreases, nausea appears, then uncontrollable vomiting and diarrhea. Adynamia intensifies, dehydration increases. In the blood, the concentration of sodium and chlorides sharply decreases and the level of potassium increases, the content of residual nitrogen increases, severe hypoglycemia is often observed, leukocytosis increases and ROE accelerates. The daily release of 17-corticosteroids, 17-hydroxycorticosteroids, and aldosterone is reduced. If untimely and irrational treatment is used, coma may develop with a fatal outcome.

Treatment

Treatment of Addisonian K. involves hormone replacement therapy, combating dehydration and electrolyte imbalance. 2-3 liters of 5% glucose solution prepared in isotonic sodium chloride solution are administered intravenously per day along with hydrocortisone in a dose of 200-500 mg or prednisolone in an amount of 50-150 mg. In combination with the above treatment, an oil solution of deoxycorticosterone is administered at a dose of 20-40 mg per day intramuscularly at intervals of 6 hours. In case of indomitable vomiting, 10% sodium chloride solution is administered intravenously in an amount of 10-20 ml. If necessary, mezaton and norepinephrine hydrotartrate are prescribed.

Hemolytic crises

Hemolytic crises are characterized by the sudden and rapid development of hemolytic anemia (see). K. may be a consequence of autoimmune processes in the patient’s body; may occur as a result of poisoning with hemolytic poisons or transfusion of incompatible blood (by Rh factor or group); can be triggered by various indifferent factors in individuals with enzymopathy (deficiency of glucose-6-phosphate dehydrogenase in erythrocytes). Hemolytic K. is characterized by acute chills and fever, severe headache, olive-yellow coloration of the skin, and severe shortness of breath. Sometimes abdominal pain occurs, reminiscent of an acute abdomen. Uncontrollable vomiting develops with huge masses of bile, often liquid feces. Urine is the color of black beer or a strong solution of potassium permanganate. In severe cases, K. may be complicated by acute renal failure.

Hemolysis develops rapidly, jaundice begins 2-3 hours after the onset of the disease and reaches a maximum after 15-20 hours. During the first 24 hours, severe normochromic anemia appears. If the course is favorable, hemolysis ends within 2-4 weeks. significant improvement or complete recovery occurs. In severe cases, death from anemic coma or uremia is possible (see).

Treatment

For autoimmune hemolytic K., the treatment of choice is glucocorticoid hormones, prescribed in large doses (prednisolone 50-100 mg per day orally). In case of acute toxic hemolytic K., with enzymopathies and paroxysmal hemoglobinuria, repeated blood transfusions of 250-500 ml are indicated, for a total of up to 1 - 2 liters per day (in the absence of signs of renal failure); intravenous administration of fluids (40% glucose solution; polyglucin) up to 400-500 ml per day; prescribing medium doses of glucocorticoids (25-40 mg prednisolone per day). An effective method of combating acute uremia is hemodialysis (see). In acute hemolytic K. caused by intracellular hemolysis (in patients with Minkowski-Choffard disease), splenectomy is indicated under the protection of a blood transfusion.

Erythremic crises

Erythremic crises occur with polycythemia (see) against the background of a sharp increase in the number of red blood cells. They are characterized by severe weakness, drowsiness, headache, vomiting, dizziness, tinnitus (wedge, the picture may resemble Meniere's syndrome). Patients feel a rush to the head, a feeling of heat. Erythremic K. essentially belong to cerebral K. They are based on a violation of cerebral hemodynamics due to erythremia, a sharp thickening of the blood.

Treatment

With erythremic K., repeated bloodletting, the use of leeches, the administration of anticoagulants, as well as symptomatic drugs are indicated.

Bogolepov N.K. Cerebral crises and stroke, M., 1971, bibliogr.; Grashchenkov N. I. and Boeva E. M. Dynamic disorders of cerebral circulation, Vestn. USSR Academy of Medical Sciences, No. 10, p. 48, 1958; Grinstein A. M. and Popova N. A. Vegetative syndromes, M., 1971; Kalinin A.P. and Lukyanchikov V.S. Hyper- and hypocalcemic crises as emergencies in pathology of the parathyroid glands, Ter. arkh., t. 50, no. 5, p. 136, 1978, bibliogr.; Kreindler A. Cerebral infarction and cerebral hemorrhage, trans. from Romanians, Bucharest, 1975, bibliogr.; Moiseev S. G. Hypertensive cardiac crises, Klin, med., t. 54, No. 2, p. 43, 1976; Transient disorders of cerebral circulation, ed. R. A. Tkacheva, M., 1967, bibliogr.; Ratner N. A., Denisova E. A. and Smazhnova N. A. Hypertensive crises, M., 1958, bibliogr.; Guide to Clinical Endocrinology, ed. V. G. Baranova, L., 1977; Schmidt E. V. Structure of transient disorders of cerebral circulation, Zhurn, neuropath, and psychiat., t. 73, no. 12, p. 1761, 1973, bibliogr.; Schmidt E.V., Lunev D.K. and Vereshchagin N.V. Vascular diseases of the brain and spinal cord, M., 1976, bibliogr.; Erina E. V. Treatment of hypertension, M., 1973, bibliogr.; Heintz R. Akute hypertensive Krisen bei essentieller und renaler Hyper-tonie, in the book: Aktuelle Hypertonieprobleme, hrsg. v. H. Losse u. R. Heintz, S. 120, Stuttgart, 1973.

D.K. Lunev, E. A. Nemchinov, M. L. Fedorova.

An acute hemolytic crisis can be caused by a hereditary predisposition of red blood cells, as well as the occurrence of immune hemolytic anemia, when antibodies destroy red blood cell cells.

Also, a crisis can occur when transfusion of blood that is incompatible with the donor, or if the material is bacterially contaminated. Red blood cells can also be destroyed when a number of blood diseases occur.

Taking certain medications (quinidine, sulfonamides, etc.) can also cause a hemolytic crisis if the patient had hereditary hemolytic anemia. Also, people susceptible to the disease include those who are exposed to intense physical activity, go in for parachuting, paragliding and mountaineering. That is, those sports in which the human body experiences a sharp change in atmospheric pressure.

Hemolytic crisis: symptoms

Hemolytic crisis can be diagnosed by a combination of several characteristic symptoms:

the person turns pale;
he is shivering;
body temperature rises sharply;
cramping pain occurs in the abdomen and lower back;
mucous membranes turn yellow.

Brain phenomena such as a sharp decrease in vision, dizziness, and even loss of consciousness also occur. The concentration of reticulocytes in the blood increases, bilirubin and free hemoglobin increase in the plasma.

Blood plasma may appear yellow or pink. The content of urea and free hemoglobin increases. Acute renal failure may develop, which can progress to complete anuria, and in some cases even to uremia.

Hemolytic crisis: emergency care

To provide first aid, it is necessary to warm the human body; for this you can use a heating pad. The use of drugs such as heparin, metipred or prednisone is very effective. They are administered intravenously.

It is necessary to carry out therapy using hormonal and antihistamine drugs. These include:

The basis for a favorable outcome after a hemolytic crisis is how quickly the patient is transported to the nearest hematology hospital, where he can receive emergency care.

During the initial examination of the patient in the hospital, the diagnosis is clarified. In severe cases, a blood transfusion is performed, for which donor blood is selected, the red blood cells of which must be completely compatible with the patient’s blood.

To do this, use a washed erythrocyte suspension, which must be prepared 5-6 days before the procedure. If the patient is found to be poisoned by hemolytic poisons, then the most effective procedure is therapeutic plasmapheresis. It allows you to very quickly cleanse the blood of the agent that caused hemolysis, as well as immune complexes and antibodies. Transfusion therapy can be carried out only after a complete examination of the patient, so as not to cause increased hemolysis.

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Hemolytic crisis

An acute hemolytic crisis can be caused by hereditary pathology of erythrocytes or destruction of erythrocytes by antibodies (immune hemolytic anemia), transfusion of incompatible or bacterially contaminated blood, intense damage to erythrocytes in various blood diseases. In a number of hereditary hemolytic anemias, acute hemolysis can be provoked by taking certain medications (sulfonamides, quinidine, etc.), heavy physical activity, large changes in atmospheric pressure (climbing mountains, flying on unpressurized airplanes and gliders, parachuting).

A hemolytic crisis is characterized by the rapid development of general weakness, cramping pain in the lower back and abdomen, chills and fever, as well as cerebral symptoms (dizziness, loss of consciousness, meningeal symptoms, blurred vision), pain in the bones and joints. General pallor appears, combined with icteric staining of the mucous membranes. During a hemolytic crisis, acute renal failure often occurs, including complete anuria and uremia. The content of hemoglobin and red blood cells in the blood decreases; the plasma may be icteric or pink. The content of reticulocytes in the blood is sharply increased; bilirubin in the blood plasma increases, as well as the level of free hemoglobin, residual nitrogen and urea.

Emergency care for hemolytic crisis. Warming the body with a heating pad, intravenous administration of prednisolone (metipred) and heparin. Treatment is carried out with antihistamines and hormonal drugs: calcium chloride or gluconate, promedol. Rapid delivery of the patient to a hematology hospital, where the diagnosis is clarified and, if blood transfusion is necessary, compatible donor red blood cells are selected. The latter are administered in the form of a washed erythrocyte suspension, preferably after 5-6 days of storage. In case of poisoning with hemolytic poisons, therapeutic plasmapheresis is indicated to quickly remove the agent, antibodies and immune complexes from the blood that caused hemolysis. Transfusion therapy should be carried out for health reasons with great caution, as it can increase hemolysis and provoke a second wave.

Hematology-Hemolytic crisis

Hemolytic crisis occurs due to severe hemolysis of red blood cells. Observed in congenital and acquired hemolytic anemia, systemic blood diseases, transfusion of incompatible blood, the action of various

hemolytic poisons, as well as after taking a number of medications (sulfonamides, quinidine, the nitrofuran group, amidopyrcca, resokhin, etc.).

The development of a crisis begins with the appearance of chills, weakness, nausea, vomiting, cramping pain in the abdomen and lower back, increasing shortness of breath, increased body temperature, icterus of the mucous membranes and skin, and tachycardia.

During a severe crisis, blood pressure drops sharply, collapse and anuria develop. An enlargement of the spleen and sometimes the liver is often observed.

Characteristics: rapidly developing severe anemia, reticulocytosis (reaching), neutrophilic leukocytosis, increased levels of indirect (free) bilirubin, often positive Coombs tests (with autoimmune hemolytic anemia), the presence of urobilin and free hemoglobin in the urine (with intravascular hemolysis).

Differential diagnosis is carried out between diseases leading to the development of hemolysis (congenital and acquired hemolytic anemia), as well as post-transfusion hemolysis, hemolysis due to the action of hemolytic poisons and certain medications.

With congenital hemolytic anemia, an enlarged spleen, reticulocytosis, microspherocytosis, a decrease in the osmotic resistance of red blood cells and a high level of indirect bilirubin in the blood are determined.

In the diagnosis of autoimmune hemolytic anemia, anamnesis data (duration of the disease, the presence of a similar disease in close relatives), as well as positive Coombs tests and acid erythrogram parameters are important.

The diagnosis of hemolytic crisis due to transfusion of incompatible blood is based on the anamnesis, determination of the blood group of the donor and recipient, as well as an individual compatibility test.

A history of contact with toxic substances or taking medications that can cause hemolysis, as well as the absence in patients of erythrocyte antibodies, microspherocytosis and a decrease in the level of glucose-6-phosphate dehygenase, give reason to believe that a hemolytic crisis has developed due to the action of hemolytic poisons or the toxic effects of medications.

A set of urgent measures

Therapy with antihistamines and hormonal drugs: intravenously inject 10 ml of a 10% solution of calcium chloride or calcium gluconate, subcutaneously inject 1-2 ml of a 1% solution of diphenhydramine, 1 ml of a 2% solution of promedol and intravenous prednisolone.

Administration of vasoconstrictive drugs and cardiac glycosides: intravenous drip of 1 ml of 0.06% solution of corglyakon per 500 ml of 5% glucose solution or isotonic sodium chloride solution; s/c or intravenously 1-2 ml of mezatone solution.

For the prevention of acute renal failure, intravenous drip administration of 4-5% sodium bicarbonate solution is indicated; if it develops, measures aimed at improving renal function are taken (see p. 104).

In case of post-transfusion hemolysis, prescribe a perinephric blockade and administer mannitol intravenously at the rate of 1 g per 1 kg of body weight.

In case of repeated hemolytic crises occurring with predominant intracellular hemolysis, splenectomy is indicated.

Scope of medical activities in units and military medical institutions

In the MPP (military hospital). Diagnostic measures: general blood test, urine test.

Therapeutic measures: intravenous administration of calcium chloride or calcium gluconate; subcutaneous administration of promedol with diphenhydramine and cordiamine. In case of collapse, administration is indicated. ezathone and caffeine. Evacuation of the patient to the medical center and hospital by ambulance on a stretcher, accompanied by a doctor (paramedic).

In the medical center or hospital. Diagnostic measures: urgent consultation with a therapist and surgeon, clinical analysis of blood and urine, examination of free hemoglobin in urine, determination of direct and indirect bilirubin in the blood; erythrogram, immunological reactions (Coombs tests).

Therapeutic measures: carry out therapy aimed at compensating for cardiovascular insufficiency, cor glycon, mesatone, norepinephrine; prescribe broad-spectrum antibiotics; administer intravenous pre-nisolone; if therapy is ineffective, splenectomy is performed. If acute renal failure develops, carry out a set of measures indicated in the section on the treatment of acute renal failure.

Emergency care for hemolytic anemia

In all forms of hemolytic anemia during periods of severe hemolytic crises, emergency measures are necessary aimed at neutralizing and removing toxic products from the body, preventing renal block, improving hemodynamics and microcirculation, impaired as a result of spasm of peripheral vessels, blockage of them with stromal elements and microemboli. For this purpose, in case of massive hemolysis, 5% glucose solution, Ringer's solution or isotonic sodium chloride solution 500 ml, hemodez, polyglucin or rheopolyglucin 400 ml per day, 10% albumin solution 100 ml are prescribed intravenously. To prevent the formation of hematine hydrochloride in the renal tubules, alkaline solutions are administered intravenously (90 ml of 8.4% sodium bicarbonate solution, mixed with 2-4% sodium bicarbonate solution again until an alkaline urine reaction appears - pH 7.5-8).

Cardiovascular drugs are prescribed according to indications (caffeine, corazol, etc.), as well as drugs that stimulate diuresis (intravenously, 5-10 ml of a 2.4% solution of aminophylline, 2-4 ml of a 2% solution of Lasix, 1-1 .5 g/kg mannitol in a 10-20% solution). The latter should not be used for anuria to avoid hypervolemia and tissue dehydration.

In case of no effect and worsening renal failure, hemodialysis using an artificial kidney apparatus is indicated.

In case of severe anemia, transfusions of red blood cells, washed and thawed red blood cells, individually selected according to the indirect Coombs test are performed.

During the process of hemolysis, a symptom of hypercoagulation with thrombus formation may develop. In these cases, the use of heparin according to the regimen is indicated.

Patients with acquired hemolytic anemia (especially of immune origin) are prescribed corticosteroids (prednisolone at the rate of 1-1.5 mg per 1 kg of body weight orally or parenterally, followed by a gradual reduction in dose or hydrocortisone intramuscularly). In addition to glucocorticoids, in order to relieve an immunological conflict, you can use mercaptopurine, azathioprine (imuran) pomg (1-3 tablets) per day for 2-3 weeks under the control of blood counts.

In case of Marchiafava-Micheli disease during a period of severe hemolytic crisis, anabolic hormones (methandrostenolone or nerobol) pomg per day, retabolil 1 ml (0.05 g) intramuscularly, antioxidants (tocopherol acetate 1 ml% solution, aevit 2 ml 2 times) are indicated per day intramuscularly). As blood transfusion agents, red blood cells with a shelf life of 7-9 days are recommended (during which properdin is inactivated and the risk of increased hemolysis is reduced) or red blood cells washed three times with isotonic sodium chloride solution. Laundering helps remove properdin and thrombin, as well as leukocytes and platelets that have antigenic properties. For thrombotic complications, anticoagulants are prescribed. Corticosteroids are not indicated.

For patients with hemolytic anemia, due to the possibility of developing hemosiderosis due to hemolysis and frequent blood transfusions, intramuscular administration of deferoxamine (desferol) is recommended - a drug that fixes excess iron reserves and removes them, 1-2 times a day.

For Minkowski-Choffard disease and autoimmune hemolytic anemia, splenectomy has a good effect.

Hemolytic crisis. Diagnostics and emergency care;

Treatment of hemolytic crisis in enzymatic erythropathy

(symptomatic taking into account etiopathogenesis):

Prednisolone - 2-3 mg/kg/day - first intravenously, then orally until the reticulocyte count normalizes

Transfusion of washed red blood cells with a hemoglobin content below 4.0 mmol/l (6.5 g/%), (transfusion of red blood cells without selecting an individual donor is dangerous)

Prevention of hypothermia in the presence of cold autoAT

Splenectomy in chronic cases (if corticosteroid therapy is ineffective for 6 months)

1. Elimination of the action of the etiological factor

2. Detoxification, disaggregation, anti-shock measures, fight against acute renal failure

3. Suppression of antibody formation (during immune genesis).

4. Replacement blood transfusion therapy.

5. Gravity surgery methods

First aid

Rest, warming the patient, hot sweet drink

For cardiovascular failure - dopamine, adrenaline, oxygen inhalation

For severe pain, IV analgesics.

In case of autoimmune HA, transfusions of blood incompatible with blood group and Rh factor, it is advisable to administer drugs

In case of immune genesis of hemolysis (including post-transfusion) - prednisolone 90-200 mg IV bolus

and specialized medical care