What do people die from with aplastic anemia? Failure of vital organs

This is a hematological syndrome caused by the action of a large number of exogenous and endogenous factors. Its characteristic feature is qualitative and quantitative changes in hematopoietic cells and their microenvironment with the development of pancytopenia in the peripheral blood, hypocellularity and fatty infiltration of the bone marrow.

Two age peaks of the incidence of aplastic anemia are determined - 20 and 65 years. In this regard, there is an opinion about the possible etiological role of viruses (at a young age) and chemicals (at the elderly). However, most authors tend to consider aplastic anemia as a polyetiological disease.

probable cause of aplastic anemia found in approximately 50% of patients. Possible etiological factors include:

certain medications (antibiotics, sulfa drugs, gold preparations, non-steroidal anti-inflammatory and sedatives, tranquilizers, cytostatics, etc.),
chemicals (mercury vapor, acids, dyes, varnishes, paints, household chemicals, gasoline, benzene, ursol, etc.),
physical factors (radionuclides),
infectious agents (hepatitis B and C viruses, retroviruses, mycobacterium tuberculosis, etc.),
autoimmune processes and diseases (systemic lupus erythematosus, Sjögren's syndrome, eosinophilic fasciitis, etc.).

In the development of aplastic anemia, genetic predisposition and individual reactivity of the organism matter. Three main concepts of possible mechanisms for the occurrence of bone marrow hematopoiesis disorders in aplastic anemia are considered:

damage to hematopoietic stem cells
violation of immunological control over the processes of hematopoiesis (cellular and humoral),
defective hematopoiesis of the microenvironment.

The first clinical manifestations of aplastic anemia are different. Acute onset is observed in 12-15% of patients. It is characterized by the rapid development of anemia, severe hemorrhagic syndrome, often fever with chills. Acute onset is usually observed in young people. More often, aplastic anemia develops gradually, manifested by anemic and hemorrhagic syndromes. The course is determined in 85% of patients with aplastic anemia.

The reason for the initial visit to the doctor may be increasing general weakness, lethargy, pallor, the appearance of shortness of breath and palpitations when walking, pain in the heart as a sign of anemic hypoxia. Sometimes there are hemorrhagic manifestations of varying degrees and localization - bleeding from the nose and gums, skin hemorrhages (from small to continuous), menorrhagia. Often there is hematuria, bleeding from the digestive tract.

In 84% of patients, retinal hemorrhages are detected during ophthalmoscopy. Fever is often observed due to necrotic changes in the mucous membranes of the oral cavity, digestive tract, suppuration of extensive hemorrhages in internal organs and tissues. Thus, the clinical manifestations of aplastic anemia are directly related to changes in the peripheral blood (anemia, leukopenia, thrombocytopenia) and cover, respectively, lean, hemorrhagic syndromes and infectious complications syndrome.

Splenomegaly, hepatomegaly, enlarged lymph nodes are not typical for aplastic anemia. An increase in the size of the liver can be observed only in persons who have had hepatitis, and the spleen in patients who have repeatedly undergone blood transfusions. Aplastic anemia often develops over a long period of time (months and years), may be accompanied first by the reduction of one of the hematopoietic sprouts with gradual changes in all sprouts.

How to treat aplastic anemia?

Treatment of aplastic anemia remains an unresolved problem, since there is no fundamental knowledge about its pathogenesis, there is a high risk of fatal complications of treatment in severe forms of the course. Most modern therapies are developed primarily on the basis of empirical research results.

The two leading directions in treatment of aplastic anemia are measures aimed at restoring bone marrow hematopoiesis and preventing hemorrhagic and infectious complications and manifestations of anemia. Treatment should begin immediately after diagnosis.

The use of cyclosporine A allows you to get a response in almost a third of patients refractory to therapy with antilymphocyte globulin. It is assumed that the mechanism of action of cyclosporine A is associated with an irreversible and specific effect on the stem cell genome, with inhibition of apoptosis, inhibition of the production of interleukin-2. The drug does not affect the macrophage link of immunity and, as a result, does not reduce the body's resistance to infections.

During the first 8-10 days, antilymphocyte and antiplatelet globulin are prescribed in parallel as a long-term infusion.

Of the side effects of the use of these drugs - a high frequency of allergic reactions of the immediate (anaphylactic shock) and delayed (serum sickness, urticaria, myalgia, arthralgia, hyperthermia) type.

Corticoids have long been considered as one of the effective treatments for aplastic anemia. However, it has been proven that remission after corticoid therapy is observed in only 12% of patients, and the combination of corticoids with androgens does not improve treatment outcomes. High doses of methylprednisolone may be recommended as an alternative form of immunosuppressive therapy in severely ill patients. The high frequency of complications does not always allow its use.

Today, bone marrow transplantation is the only radical treatment for aplastic anemia, since in some cases it can restore normal hematopoiesis. Replacement therapy with blood components is carried out according to reasonable indications for its appointment. Indications for transfusion of washed erythrocytes:

hemoglobin level below 80 g/l;
hematocrit less than 30%.

The threat of systemic or local infection is determined by the level of granulocytes. A high risk factor for infection should be considered a granulocyte level below 0.2*10 9 /l, a moderate risk factor - a granulocyte level of 0.2-0.5*10 9 /l and a low risk factor - more than 0.5*10 9 /l. To reduce the risk of hemorrhages and local inflammatory processes, intramuscular and subcutaneous injections are not carried out.

In the event of a fever, patients are prescribed empirical antibiotic therapy with antibiotics, primarily suppressing the gram-negative microflora.

The use of androgens as an independent method aplastic anemia treatment may be justified in its chronic non-aggressive course. Most often, testosterone propionate, methyltestosterone, oxymetholone are used at an initial dose of 1-2 mg/kg body weight per day. Treatment lasts an average of 1-3 months. Splenectomy as a method of treatment of aplastic anemia is almost not used in recent years.

What diseases can be associated

Differential diagnosis is carried out with diseases that are accompanied by pancytopenia. In clinical practice, it is most often necessary to differentiate aplastic anemia from, and. The clinical picture of acute leukemia, which debuts with pancytopenia, sometimes does not differ from that of aplastic anemia. Bone marrow aspiration biopsy and trepanobiopsy allow to verify the diagnosis of aplastic anemia. In acute leukemia, the spleen may enlarge and cytogenetic abnormalities may appear. Hairy cell leukemia may be accompanied by bone marrow aplasia, but more often by splenomegaly.

The greatest difficulties arise in differential diagnosis when there is no splenomegaly. Trepanobiopsy reveals an increase in the number of reticulin fibers and replacement of myeloid tissue with amorphous eosinophilic material. Application of the method of flow cytophotometry allows to reveal the expression of receptors.

Myelodysplastic syndrome is sometimes difficult to distinguish from aplastic anemia. The only method that allows differential diagnosis in complex cases is ultrasound. Cytogenetic abnormalities are indicative of myelodysplastic syndrome.

It happens in children 5-12 years old. The disease debuts with thrombocytopenia and anemia. There are two types of Fanconi anemia - the first (classic) with gross malformations of the skeleton and internal organs, and the second affects small anomalies, hyperpigmentation of skin areas, growth retardation.

Aplastic anemia can be complicated by a severe form of steroid diabetes mellitus, infectious complications, in particular against the background of inadequately selected therapy.

Treatment of aplastic anemia at home

Treatment of aplastic anemia usually requires hospitalization, at least initially. Subsequently, the patient can be transferred to outpatient treatment or dispensary observation. Compliance with all medical prescriptions, good nutrition, lack of mental and physical exertion is required.

What drugs are used to treat aplastic anemia?

The dose used for treatment of aplastic anemia ranges from 5-10 mg/kg of body weight per day in two doses, it is regulated by the level of creatinine in the blood serum. The duration of the course is on average 3-10 months (but not less than 3 months).

Assign at a dose of 15 mg/kg of body weight per day as a long-term, 8-12-hour infusion every day for 8-10 days.

Antiplatelet globulin is prescribed at a dose of 0.75 mg/kg of body weight per day as a long-term, 4-5-hour infusion every day for 8-10 days.

Assign high doses (0.5-1 g per day) intravenously for 5 days.

Assign an initial dose of 1-2 mg / kg of body weight per day; treatment lasts an average of 1-3 months.

Treatment of aplastic anemia with folk methods

aplastic anemia- one of the most severe disorders of hematopoiesis with a mortality rate exceeding 80%. This alone serves as a contraindication to all kinds of experiments in treatment. Self-medication is unacceptable, a variety of which traditional medicine usually becomes. Deterioration of well-being akin to those observed in aplastic anemia should be a reason for immediate medical attention for professional help.

Treatment of aplastic anemia during pregnancy

Treatment of aplastic anemia during pregnancy is a complex and time-consuming process. Modern medicine does not have a wide arsenal of remedies that restore health in this case, and therefore pregnant women are treated according to the standard scheme.

Such a diagnosis during pregnancy requires the entire spectrum of differential diagnosis and the absolute certainty of the doctor that it is this type of anemia that has developed in the expectant mother. It is not excluded that the question of maintaining pregnancy is raised, because the treatment and the aggressive disease itself affect the formation of the fetus.

Which doctors to contact if you have aplastic anemia

The diagnosis of aplastic anemia is mainly verified by the results of a study of peripheral blood and bone marrow. In the analysis of peripheral blood of patients with aplastic anemia, normochromic anemia of varying severity and hemoglobin concentration are found. Against the background of anemia, leukopenia develops with granulocytopenia and relative lymphocytosis. Thrombocytopenia is often profound, sometimes up to single platelets in blood smears.

A significant increase in ESR is observed, especially with severe anemia, foci of necrosis and massive hemorrhages. A feature of aplastic anemia is a decrease in the number of reticulocytes in the peripheral blood. The appearance in the peripheral blood of erythrokaryocytes (normoblasts), morphologically altered erythrocytes and leukocytes is not typical for aplastic anemia.

The content of iron in the blood serum of patients with aplastic anemia is normal or slightly elevated. With the help of an aspiration biopsy of the bone marrow, a decrease in the total number of myelokaryocytes is detected, as well as inhibition of all germs of hematopoiesis.

In the myelogram, significant disturbances in the bone marrow hematopoiesis are found. Attention is drawn to the decrease in the total percentage of cellular elements of granulocytopoiesis due to a decrease in the number of both young forms of neutrophilic cells and mature granulocytes. An increase in the relative content of lymphocytes and plasma cells in the bone marrow is often noted. A delay in the maturation of erythroid cells at the stage of polychromatophilic normoblasts (erythrocaryocytes) is observed. At the same time, the activity of erythrocytosis in general, according to the myelogram, can be both reduced and somewhat increased. Megakaryocytes are found in small numbers, often with degenerative changes, disorders of platelet lacing. Fat cells and elements of stromal origin are evident in punctate in significant quantities.

Histological preparations of trepanobioptates from patients with aplastic anemia show bone marrow aplasia with replacement of hematopoietic tissue with adipose tissue. Under the condition of effective treatment in the bone marrow, the restoration of hematopoiesis begins with an erythroid germ. Later, granulocytopoiesis is restored and, last of all, the megakaryocytic sprout regenerates.

For patients with severe aplastic anemia, trepanobiopsy is performed in a hospital setting, and for patients with a non-severe course, on an outpatient basis or in a day hospital.

Immunological studies in aplastic anemia are carried out to determine blood groups and Rh affiliation, as well as to conduct HLA typing when selecting bone marrow for transplantation.

Virological studies in aplastic anemia are reduced to the detection of antibodies to cytomegalovirus infection (CMV), since patients "seronegative" for CMV infection should receive CMV-negative blood components.

If necessary, markers of hepatitis are determined, primarily hepatitis B and C. Ultrasound of the abdominal cavity and retroperitoneal space reveals kidney anomalies, along with x-ray changes in the bones of the forearm and hands in children and young people, may indicate Fanconi anemia - a hereditary form of aplastic anemia. The presence of spleno- and hepatomegaly may indicate hemoblastosis.

Cytogenetic studies in difficult diagnostic cases help to reject myelodysplastic syndrome. Typical cytogenetic anomalies for Fanconi anemia.

Criteria for the diagnosis of aplastic anemia:

hemoglobin concentration less than 100 g/l or hematocrit less than 30%.
the number of leukocytes in the peripheral blood is less than 3.5*10 9 /l, the number of granulocytes is less than 1.5*10 9 /l.
the number of platelets in the peripheral blood is less than 50*10 9 /l.

The diagnosis of aplastic anemia is considered reliable in the presence of two of the three named criteria in combination with hypocellular bone marrow in the absence of blast forms in the peripheral blood and in the bone marrow. Depending on changes in the peripheral blood and myelogram, the following clinical forms of anemia are distinguished:

with a mild course;

with a severe course;
with super heavy duty.

Aplastic anemia with a non-severe course has the following laboratory and hematological characteristics:

in the peripheral blood, the number of granulocytes is more than 0.5 * 10 9 / l,
platelets - 20-50*10 9 /l,
reticulocytes - more than 1%,
in the bone marrow (according to bone marrow aspiration biopsy and trepanobiopsy), zones of preserved hematopoiesis are determined near areas of reduced cellularity,
the content of non-myeloid cells is less than 50%,
according to trepanobiopsy, signs of bone marrow hypoplasia are observed.

Severe aplastic anemia is characterized by:

a decrease in the number of granulocytes in the peripheral blood (less than 0.5 * 10 9 / l),
a decrease in the number of platelets in the peripheral blood (less than 20 * 10 9 / l),
a decrease in the number of reticulocytes in the peripheral blood (less than 1%),
in the bone marrow - a decrease in the number of myelokaryocytes (less than 40 * 10 9 / l),
the content of non-myeloid cells - more than 50%,
according to trepanobiopsy, they show bone marrow aplasia.

For aplastic anemia with a super-heavy course, the characteristic features are.

Aplastic anemia is a blood disease characterized by a decrease in the hematopoietic function of the bone marrow and a decrease in the production of blood cells by it. This pathology is rare, but has a severe course and in many cases leads to the death of the patient.

Aplastic anemia is an insidious pathology, since it does not make itself felt for a long time, and when the symptoms become apparent, the disease becomes extremely dangerous and even incurable. Therefore, doctors recommend that all people undergo regular examinations to identify such insidious pathologies at an early stage of development. People of any age and gender, including children, suffer from this pathology.

Etiology

Many are interested in the question, what is aplastic anemia and why does it appear? Unfortunately, modern medicine cannot name the obvious reasons for the development of aplastic anemia in humans. But it is known that the disease is acquired and hereditary.

There is a theory that the development of pathology is associated with the characteristics of the functioning of the body of each individual. However, there are some factors that can trigger the onset of the disease. In particular, the clearest of the factors is the impact on a person of ionizing radiation, which suppresses the functions of the bone marrow and leads to a decrease in their production, and. Other predisposing factors include:

poor ecological situation in the region;
regular human contact with harmful chemicals;
some infectious pathologies, in particular;
problems with the bone marrow;
taking certain medications, including common antipyretics and pain relievers such as aspirin;
frequent use of antibiotics, especially chloramphenicol.

The disease has been found to be observed in patients with excessive alcohol cravings. Genetic predisposition also plays an important role in the development of the disease. Aplastic anemia in children often develops due to hereditary pathologies, including Fanconi anemia. It should be noted that often children are diagnosed with idiopathic aplastic anemia - that is, a pathology with an unclear etiology.

As for such a pathology as hypoplastic aplastic anemia, this is an even more severe pathology that causes serious disturbances in the work of all internal organs and leads to a disruption in the performance of body systems.

It should also be said that aplastic anemia can be of three forms of severity:

light;
middle;
heavy.

Symptoms

As mentioned above, aplastic anemia is an insidious pathology, the causes of which are not fully understood. In the initial stages, it does not manifest itself in any way - people note only general symptoms, which are characterized by:

fast fatigue;
pallor of the skin;
the occurrence of shortness of breath during physical exertion;
decrease in concentration.
sometimes there is tinnitus and dizziness.

People attribute all these symptoms to a decrease in tone and. However, in fact, they can be harbingers of a serious illness, so you should immediately consult a doctor for an examination.

If we talk about the later symptoms of aplastic anemia, then these include:

bleeding gums;
fever;
frequent bleeding from the nose;
tendency to frequent infectious diseases.

This symptomatology suggests that not only the synthesis of erythrocytes, but also leukocytes and platelets is impaired in the bone marrow. In addition, there are changes in the blood, which can be detected after passing certain tests.

Diagnostics

To establish a diagnosis, the patient requires a thorough examination and delivery of certain tests. In particular, patients are prescribed, in which a decrease in the number of blood cells can be seen. A bone marrow biopsy is also prescribed to clarify the stage of the disease and determine the number of blood cells in it. With this pathology, it is determined that most of the bone marrow has been replaced by adipose tissue, which is why the organ cannot cope with the function of hematopoiesis.

Unfortunately, the prognosis of the disease is unfavorable - without appropriate treatment, in 90 cases out of 100, the patient dies within one year, however, timely treatment can prolong the life of the patient and even completely cure him of the disease. The most favorable prognosis is in children and young people whose body is still capable of self-healing.

In the case of diagnosing aplastic anemia in children, it is important to determine whether it is a hereditary form - Fanconi anemia, since the treatment of this disease differs from the treatment of the classical form of pathology. To determine the form, cytogenetic studies are carried out.

Treatment

Treatment of this pathology should begin as early as possible, until the bone marrow has completely stopped producing blood cells. Patients with this diagnosis are prescribed a blood transfusion, which allows you to make up for the lack of blood cells in the body. However, it should be understood that such therapy is not a panacea and only allows you to eliminate the symptoms, and has a supporting effect.

Be sure to make sure that the pathology is not caused by a certain adverse effect - if so, adverse factors must be eliminated before starting treatment.

Patients are also prescribed immunosuppressants - these drugs disarm the immune system, preventing it from destroying brain cells, which gives the bone marrow a chance to restart the process of hematopoiesis. To eliminate the side effects of taking immunosuppressants, people are often prescribed concomitant steroids. And since the drugs are administered intravenously, and the procedure is controlled by a doctor, hospitalization of the patient in a hospital is indicated at this stage.

The most effective treatment for a pathology such as aplastic anemia is a bone marrow transplant. This treatment is indicated even for those patients who have a severe form of pathology. At the same time, it is important that the transplant taken from the donor, according to five or more criteria, corresponds to the bone marrow of the recipient. Otherwise, transplant rejection is possible.

In the process of treatment, it is also very important to protect the body from the possible penetration of fungi and bacteria, for which patients are prescribed appropriate antifungal and antibacterial drugs.

Is everything correct in the article from a medical point of view?

Answer only if you have proven medical knowledge

Diseases with similar symptoms:

Inflammation of the lungs (officially pneumonia) is an inflammatory process in one or both respiratory organs, which is usually infectious in nature and is caused by various viruses, bacteria and fungi. In ancient times, this disease was considered one of the most dangerous, and although modern treatments allow you to quickly and without consequences get rid of the infection, the disease has not lost its relevance. According to official figures, in our country every year about a million people suffer from pneumonia in one form or another.

Today, there are many different disorders in the functioning of the human circulatory system, which have different causes from each other, manifest themselves clinically in different ways. The prognosis of the course of such diseases is also different. One example of such diseases is aplastic anemia.

Peculiarities

When a person first encounters the diagnosis of aplastic anemia, naturally, he immediately has a question, what is it? The basis of this disease (its other name is panmyelophthisis) is a sharp inhibition of hematopoiesis in the red bone marrow, which is clinically manifested in a decrease in the content of its uniform cells in the blood - erythrocytes, leukocytes and platelets.

The percentage of detection of aplastic anemia among the population is relatively low and the frequency of detected cases is only 0.5 per 100 thousand of the population. This does not depend on the sex of the sick person, but it is possible to establish some dependence on age.

From birth to age 20, the frequency of detected cases among this age group gradually increases; in the category of patients aged 20 to 55 years, it remains at the same level, but after 55 years it increases sharply.

According to recent research in this area, a small group of patients have been found to be genetically predisposed to developing aplastic anemia.

Death occurs in more than half of the patients. Some sources note that this percentage reaches 80.

Kinds

According to the ICD 10th revision, aplastic anemia is included in the "other anemia" group under the code D61.

Depending on the clinical picture, the manifestations of the disease are distinguished:

Hereditary aplastic anemia. These pathologies lead to a total defeat of the hematopoietic system. Among this type of anemia, there are two subspecies:

Fanconi anemia - if the underlying disease is accompanied by the formation of congenital malformations;
Estren-Dameshek anemia - if there are no congenital malformations;
Diamond-Blackfan anemia is a form of anemia in which there is a violation of the production of only red blood cells due to genetic factors.

Acquired aplastic (or hypoplastic) anemia. There are also subspecies here:

forms of anemia, which are characterized by an acute, subacute or chronic process of inhibition of general hematopoiesis;
partial (red cell) anemia - the process of formation of only red blood cells is inhibited.

The reasons

The causes of aplastic anemia are as follows:

The presence of external factors that have a myelotoxic effect, that is, they provoke cytostatic ones. This includes various diseases of an infectious and viral nature, and the effect of ionizing radiation, and some drugs (analgin, anti-tuberculosis drugs, some types of antibiotics), as well as drugs used in chemotherapy.
Endogenous, that is, internal, causes of aplastic anemia - the accumulation of toxic substances as a result of internal disorders and endocrine changes, for example, in the case of the development of hypothyroidism, uremia.
Autoaggression, when the patient develops individual sensitivity to antigens and a.
Idiopathic forms of aplastic anemia. They are distinguished in half of the patients, they are diagnosed if it was not possible to establish the cause of the development of the disease.

At the present stage, specialists managed to study to the greatest extent only congenital types of aplastic anemia. So, in the case of diagnosing Fanconi anemia, the reason is changes in paired chromosomes I and VII. In Diamond-Blackfan anemia, the genes of chromosomes I, XVI, XIX, and XIII are mutated. The impact on the body of free radicals can play a role in these processes.

Modern medicine is not yet fully aware of the mechanisms and causes that lead to the formation of underdevelopment of the bone marrow.

There are several processes of development of aplastic anemia:

The development of processes in the body that lead to damage to bone marrow stem cells.
The action of the body's defense mechanisms (cellular, under the influence of hormones), as a result of which the formation of blood cells is suppressed.
Various types of dysfunctions of the elements of the microenvironment of the bone marrow (osteogenic, fat cells, macrophages, and others).
Lack of factors in the body that activate the processes of hematopoiesis.
Cases when the concentration of substances necessary for hematopoiesis is maintained at the proper level (in particular, vitamin B12, protoporphyrin), but they are not absorbed by the hematopoietic tissue.

If a person develops aplastic anemia and damage to the bone marrow, the destruction of blood cells of varying degrees of maturity occurs, but, first of all, erythrocytes suffer. Not only is it disturbed, but the duration of life of mature forms also decreases.

The disease of anemia is accompanied by violations of the processes of excretion of excess iron, which accumulates in the liver and spleen.

At stage II, patients with aplastic anemia have pallor of the skin and visible mucous membranes, and bruising can sometimes occur. If the disease becomes acute, in addition to the pallor of the skin, necrosis of the mucous membranes and high temperature are also observed. Various inflammatory processes are activated in the body (in particular, pneumonia).

Usually the liver and spleen do not increase, but if an autoimmune form of aplastic anemia is diagnosed, in which antibodies to red blood cells are produced in the patient's body, moderate splenomegaly (enlargement of the spleen) and slight yellowness of the skin and sclera may develop, caused by the presence of hemolytic components in the blood.

Indicators in the blood test

The most pronounced is the III stage of anemia, which is characterized by a stormy picture of clinical manifestations. At this stage in the development of aplastic anemia, a blood test shows:

c pronounced anemia is determined (usually normochromic) - the hemoglobin level decreases to 20 - 30 g / l, the concentration of reticulocytes decreases (indicates a decrease in the functionality of the bone marrow);
, granulocytopenia, that is, the level of granular leukocytes in the blood decreases sharply. At the same time, the number of lymphocytes does not change;
, down to zero;
histological examination of bone marrow tissue shows a catastrophic disappearance of its cells, which are replaced by adipose tissue;
sharply - up to 30 - 50 mm / hour;
serum iron concentration increases.

Treatment

Treatment of aplastic anemia depends on the main factor that allegedly caused the development of the pathological process.

If the disease is detected for the first time, the patient must be hospitalized in a hospital, hematology department. Only in the conditions of a medical institution can you correctly select the drug necessary for treatment, as well as determine its dosage.

The main methods of correction and treatment of aplastic anemia in children and adults are:

transfusion methods ();
transplant methods;
medicinal methods.

The transfusion therapeutic method involves the transfusion of a whole, erythrocyte or platelet mass to the patient, as well as. Blood products used in transfusion are prepared at transfusion stations from donated blood. This method of transfusion is temporary, since it only makes it possible to replenish the deficiency of blood cells, but the disorders in the bone marrow are not eliminated. Another disadvantage of transfusion is that it cannot be used when diagnosing autoimmune forms of aplastic anemia.

If the patient undergoes blood transfusion operations quite often, this can lead to the accumulation of excess iron in his body, which is deposited in the liver and spleen. Therefore, in this category of patients, drugs that affect the excretion of iron from the blood are included in therapy.

Transplantation

The most effective method of treating aplastic anemia is considered to be transplantation, in which a human bone marrow is transplanted. The probability of success of a bone marrow transplant depends on the age of the patient, the percentage of successful outcome is higher, the younger the patient. One of the closest relatives who has the same blood type as the patient can act as a donor. To do this, it is necessary to conduct a study on the individual compatibility of their blood groups.

This treatment technique requires some preparation in order to reduce the percentage of the likelihood of rejection of foreign tissues. Before they are canceled, radiation therapy is prescribed, followed by a course of chemotherapy drugs. This is done in order to temporarily suppress the patient's immune system, which can provoke rejection of donor stem cells. Bone marrow transplantation for anemia is a very expensive operation, performed only in specialized clinics.

Medicines

Drug therapy for aplastic anemia in humans includes the use of the following drugs:

immunosuppressants - for example, cyclosporine, specific antiglobulins. The use of these dosage forms is indicated in cases where it is impossible to perform a bone marrow transplant operation. They are prescribed in combination with corticosteroids to prevent the development of anaphylactic reactions;
drugs that stimulate hematopoietic processes - for example, filgrastim, leukomax. They are prescribed only in case of diagnosing leukopenia, since they activate the processes of formation of granular leukocytes;
for the treatment of aplastic anemia in men, drugs containing androgens (male sex hormones) are used - testosterone propionate, sustanon;
with severe hemorrhagic syndrome, the appointment of hemostatics is indicated - dicinone, aminocaproic acid;
one example of drugs that help remove iron from the blood is desferal.

Splenectomy

Another method of treating aplastic anemia is splenectomy, in other words, surgery to remove the spleen. The frequency of the positive effect is 85%. The basis of this method of treatment is the cessation of autoimmune processes in the body, when antibodies are produced to its own cells. It can be performed on any patient who does not have infectious complications.

It is forbidden to resort to the use of traditional medicine recipes in the treatment of aplastic anemia. Treatment of pathological disorders in this case provides for the exact dosage of medicinal substances that cannot be observed when using herbal remedies.

Can aplastic anemia be cured?

Since the mechanisms of development of aplastic anemia at this stage remain not fully understood, the prognosis for the treatment of aplastic anemia in most cases is unfavorable.

The highest mortality rate is observed in the category of patients with severe forms of the disease. Due to the progressive and uncorrectable underdevelopment of the bone marrow, it is not possible to resume the formation of blood cells, and this leads to the death of the patient due to generalized sepsis.

If the course of the disease is less severe, the patient has a positive reaction to donor stem cell transplantation, and the use of immunosuppressants has an effective effect - the forecasts for the transition of the disease to the stage of remission range from 50 to 90%. Drug therapy alone, without the use of surgical methods of treatment, can give a positive result in only half of the patients.

How the disease manifests itself in childhood

In the case of the development of hereditary forms of aplastic anemia, the clinical picture depends on the type of disease.

When diagnosing aplastic Fanconi anemia, a child has such congenital malformations as congenital anomalies in the development of the skeletal system (absence of the first finger on the hand, curvature or absence of the radial bones, and others), heart and kidney defects, anomalies in the development of the eyes (small eyeballs).

Symptoms of aplastic anemia in children begin to appear at the age of 4 years, very rarely - at an early age. The child has general weakness, increased fatigue, frequent headaches. Immunity is reduced, the child becomes more susceptible to colds.

Hemorrhagic syndrome is manifested by a tendency to frequent nosebleeds. Laboratory examination gives a characteristic clinical picture in blood tests. The disease becomes chronic, interrupted by periodic relapses.

The lethal outcome in this form of aplastic anemia is due to the addition of an infectious process or the development of acute blood loss due to increased hemorrhagic syndrome.

Cases of diagnosing aplastic anemia Estren-Dameshek are quite rare. These types of disease are characterized by disorders of only hematopoietic processes.

With Diamond-Blackfan anemia, the formation of only red blood cells is disrupted. There is no hemorrhagic syndrome. On examination, the specialist notes pallor of the skin, enlarged liver and splenomegaly. The concentration of platelets and leukocytes in the blood can only decrease if the spleen is severely damaged. The disease has a chronically severe course. The prognosis is extremely unfavorable. Death occurs before the age of 20.

Aplastic anemia is a blood disorder. It is characterized by a violation of the full growth of blood elements in the bone marrow. It is he who is responsible for the production of red blood cells, white blood cells and platelets.

The disease is most often diagnosed in patients older than 50 years, and it does not depend on gender. Small children are also affected by the disease. They most often find its hereditary forms. Lethal outcome occurs in 60% of cases.

Anatomical reference

The bone marrow is a tubular structure. It is localized in the depth of tubular bones. It is entrusted with one of the most important tasks of the hematopoietic system - the development of its main elements. It is impossible to imagine the full functioning of a healthy human body without red blood cells, white blood cells and platelets. Each of these cells plays a specific role:

Red blood cells are responsible for transporting carbon dioxide and oxygen. When these elements are deficient, the brain is the first to suffer. This condition is otherwise known as anemia.
Leukocytes take an active part in the fight against viral and bacterial diseases. If the amount of these elements in the blood is minimal, a person constantly suffers from inflammatory pathologies.
Platelets are responsible for the process of blood clotting. When their number is below a critical level, the patient is haunted by causeless hemorrhages.

In the case of anemia, a person experiences a deficiency of all of these cells. Hence the manifestations of the disease - infectious, anemic or hemorrhagic syndrome.

A similar clinical picture is usually diagnosed in various oncological pathologies. Therefore, aplastic syndrome should not be ignored.. With the appearance of its initial manifestations, you should immediately consult a doctor.

Main reasons

People who do not have a specialized medical education, most often do not have a correct idea of \u200b\u200bwhat kind of illness it is. Anemia is usually understood as a violation of the hematopoietic function of the bone marrow, which entails a decrease in the production of blood elements.

As a result, the main systems of the internal organs suffer. Very often, the pathological process ends in death.

What are the causes of the disease called doctors? Its etiology remains poorly understood. Therefore, the list of causes leading to the development of anemia is incomplete. This is first of all:

Radiation exposure.
Chemotherapy used to treat cancer.
Regular intoxication of the body.
Taking certain medications (such as antibiotics).
Autoimmune diseases. This is a vast group of ailments in which the body begins to mistake its own cells for foreign ones.
Viral infections (HIV, Epstein-Barr virus, cytomegalovirus).

If the cause of the disease cannot be identified, this condition is called "idiopathic anemia". The mechanism of its development remains unknown.

Among young patients, this pathological process is also common.. Immediately after the birth of a person, the development of the disease can be triggered by congenital syphilis or toxoplasmosis.

Varieties of the disease

The clinical classification of the disease distinguishes the following forms: congenital and acquired. The first group also includes anemia of Fanconi and Estrana-Dameshek.

The clinical picture of the disease is described in more detail below. Also, Diamond-Blackfan anemia should be considered a congenital form. In this case, only the erythrocyte germ is involved in the pathological process.

Acquired aplastic anemia can be acute, subacute and chronic.

The correct definition of the form of the pathological process allows you to prescribe competent therapy. And it is on this in most cases that the outcome of the disease depends.

Clinical picture

Symptoms of aplastic anemia in adults are largely determined by its form.

For example, the acute stage is characterized by a rapid course. The disease begins with the appearance of bleeding of unknown etiology. Gradually, the pathological process is supplemented by high temperature. Against this background, angina or pneumonia may develop. The quantitative composition of the main body fluid also changes.

Usually, a blood test indicates an acceleration of ESR, severe lymphocytosis. In this case, the death of the patient occurs within 1.5 months.

The subacute form is characterized by a somewhat different clinical picture. At the same time, there are no severe bleedings, and critical changes in the composition of the main body fluid appear after 3 months.

In the chronic form, the pathology progresses rather slowly. First, patients complain of a deterioration in general well-being, they develop weakness and pallor of the skin.

During a routine examination, a doctor diagnoses an enlarged spleen, and the lymph nodes are painful on palpation. In the case of competent and timely treatment, one can hope to stop the death of the bone marrow. Remission usually lasts for several years.

Features of the disease in children

Aplastic anemia in children always manifests itself in different ways. It all depends on the type of disease. For example, with Fanconi anemia, anomalies in the development of the bone apparatus are detected in a small patient. He may be missing fingers or some bones. As the disease progresses, the pathology is supplemented by heart or kidney problems.

As a rule, anemia in young patients begins to appear after 4 years. At first, the child complains of pain in the head. He becomes lethargic, refuses to play with his peers.

Parents are forced to constantly sit with their child on sick leave, because the usual SARS or flu “does not let go” of him. A lethal outcome at this age is possible only with the addition of an infectious process.

Estrena-Dameshek anemia is characterized exclusively by a violation of the qualitative composition of the blood. Diamond-Blackfan disease has slightly different symptoms. In this case, changes occur at the skeletal level. Bleeding is absent. The skin is most often pale with a grayish tint.

Diagnostic methods

If symptoms of anemia appear, you should immediately seek medical help. The best thing to do is to see a therapist. First, the doctor must examine the patient, study his history. In some cases, the specialist may ask a number of clarifying questions.

After that, they go directly to instrumental diagnostic methods.. Initially, the patient is assigned a blood test with a mandatory count of the leukocyte formula. After that, a bone marrow biopsy is recommended.

This procedure involves taking the material of the organ for its subsequent study in the laboratory. The oncological process can only be excluded through a biopsy.

The final stage of diagnosis is ultrasound, with which you can assess the size of the spleen and other organs.

If the doctor confirms the preliminary diagnosis, appropriate therapy is prescribed. This may be medication, transplantation or blood transfusion.

In each case, the treatment option is chosen by the doctor. At the same time, he must take into account the state of health of the alleged patient and a number of related factors: age, the presence of other pathologies, the severity of the disease.

Therapy with medicines

If the development of the pathological process was provoked by autoimmune ailments (this is an unspecified anemia), immunosuppressive drugs are prescribed. They help increase the activity of immune elements. Most often, "Tymoglobulin" is prescribed for this purpose. "Cyclosporine" for anemia also shows good efficacy.

Additionally, patients are usually prescribed bone marrow stimulants. Such medicines can enhance the process of hematopoiesis several times.

Among these drugs, Leykin, Neulasta and Neupogen deserve special attention. These drugs have been developed recently, so their cost remains at a fairly high level.

The pathogenesis of aplastic anemia is such that the disease process necessarily affects the immune system. As a result, the body becomes overly sensitive to various viral and fungal infections.

You can not neglect their symptoms, you should immediately seek qualified help. If the diagnosis confirms the addition of a particular infection, the doctor must prescribe the appropriate treatment.

It usually comes down to taking antibiotics..

Transplantation

Severe aplastic anemia provides only one treatment option - spinal cord transplantation. To be more precise, during the operation, its stem components are transplanted. It is used to treat patients under the age of 30 who have a donor suitable for numerous parameters. The role of the latter is often played by a sister or brother.

After finding and agreeing on a donor, the patient’s body begins to be “depleted” with chemotherapy. The healthy stem cells from the donor are then filtered and transplanted. In the body of a sick person, these elements independently migrate and take root.

The procedure is expensive and requires a long hospital stay. After it is carried out, the patient is forced to take medication for some time in order to prevent rejection of donor elements.

Transplantation carries some risks. Sometimes the patient's body does not accept donor cells. In this case, a relapse of the disease occurs, which in some cases ends fatally.

Blood transfusion

Treatment of aplastic anemia, which is accompanied by low platelet counts, involves a blood transfusion. This approach does not completely get rid of the disease, but helps to control its manifestations.

Despite all the positive aspects of this process, it can be accompanied by certain complications. For example, the poured mass contains a large amount of iron.

Accumulating in the body, this substance can adversely affect the work of some internal organs, damaging them. In this case, the patient is additionally prescribed drugs that help to remove excess iron.

Expectations after treatment

Most often, with this disease, the prognosis for recovery is unfavorable. As a rule, we are talking about stopping unpleasant symptoms and alleviating the suffering of the patient.

Among the main factors that increase the likelihood of a favorable outcome are:

low degree of severity of the pathological process;
competent selection of therapy and concomitant medications;
the young age of the patient (the younger the patient, the higher his chances of a complete cure).

The question of whether aplastic anemia can be cured cannot be answered unambiguously. It all depends on a number of factors.

Prevention methods

Primary is designed for a relatively healthy person. To prevent the development of anemia, you need to eat right and engage in feasible sports. Do not forget about regular procedures to strengthen the immune system. It is also necessary to periodically undergo preventive examinations by a therapist, and if ailments are detected, immediately begin treatment.

Secondary prevention is carried out to slow the progression of an already confirmed disease. To this end, it is necessary to take the prescribed medications, carefully monitor your health, and if new symptoms appear, immediately seek medical help.

Aplastic anemia (anemia) is a pathological condition characterized by inhibition of a number of functions of bone marrow stem cells related to the formation of blood cells - erythrocytes, platelets and cells of the granulocytic group.

It is classified as a disease of the blood system.

Classifications of aplastic anemia

To date, clinicians use several classifications that take into account the various parameters of this disease.

Classification according to ICD-10

Depending on the causes that caused the disease, the following forms are distinguished:

Constitutional. Hereditary forms of aplastic anemia also belong to this class;
Medical. When classifying according to ICD-10, each damaging pharmaceutical product is assigned a separate code.
Developed as a result of exposure to external causes, excluding medication.
Idiopathic aplastic anemia. With idiopathic anemia, the causes that caused the disease remain unknown.
Other forms of aplastic anemia with specified etiological factors.
Unspecified form.

Clinical classification

This gradation subdivides all cases of aplastic anemia into hereditary and acquired.

Hereditary cases of aplastic anemia with complete damage to the germ of hematopoiesis.

Anemia Fanconi. Inhibition of the hematopoietic germ in this disease is combined with.
Estren-Dameshek anemia is characterized by an isolated lesion of the hematopoietic system. There are no developmental defects.

Hereditary Dimod-Blackfen anemia. Inhibition of hematopoiesis in this disease is characterized by an isolated lesion of the erythrocyte germ.

Acquired aplastic anemia (hypoplastic anemia).

Inhibition of the general synthesis of blood cells of acute, subacute and chronic course.
An isolated lesion of only an erythrocyte germ; in these cases they speak of partial, or red cell anemia.

Etiology

According to modern statistics, in 22–51% of cases, the specific causes of the development of the disease remain unknown.

The etiology of congenital forms of aplastic anemia was most accurately established.

The etiological factors leading to the development of aplastic anemia are usually divided into two groups.

exogenous

Chemicals - petroleum products and products of their processing, mercury, benzene.
Physical factors - the negative impact of penetrating radioactive radiation.
Pharmacological substances - drugs used against tuberculosis, analgin, cytostatic agents, sulfanilamide preparations, some antibiotics and agents for the treatment of oncological pathology.
infectious agents. There is proven data on the relationship between the development of aplastic (hypoplastic) anemia and previous infectious diseases (flu, tonsillitis). In addition, the herpes, Epstein-Barr, hepatitis C, cytomegalovirus viruses have a depressing effect on the development of blood cells.

Endogenous

Endocrine disorders - such as hypothyroidism or cystic ovarian pathology in women.
Dysfunction of the immune system occurs due to the cessation of the regulatory function of the thymus in old age.

Clinical picture

congenital forms

With hereditary aplastic anemia, the symptoms depend on the disease that caused the disease. At the same time, aplastic anemia in children is, in most cases, the debuts of congenital forms of this disease.

Fanconi anemia is a hereditary pathology, which is characterized by an autosomal recessive type of inheritance. The disease usually debuts at the age of 4 to 12 years, however, hematological disorders can occur immediately after the birth of a child. With Fanconi anemia, congenital defects of the musculoskeletal system take place:

deformation or absence of the radius bones;
absence of the first fingers of the hands;
headache;
fast fatiguability;
tendency to hemorrhages (often they are the causes of death);
frequent colds;
underdevelopment of the complex of the genitourinary organs;
pronounced inhibition of all types of hematopoiesis;
when examining the bone marrow, the picture is aplastic.

Pathology usually proceeds rapidly. In the absence of timely adequate treatment, severe anemia rapidly develops, which can lead to death.

The cause of death in most cases is hemorrhage (gastrointestinal bleeding or cerebral hemorrhage).

For Fanconi anemia, the only effective treatment is bone marrow transplantation from a healthy donor, which may be, for example, a close relative of the patient.

Estrana-Dameshek anemia is characterized only by disturbances in the composition of the blood.

Diamond-Blackfan anemia is a disease from the category of hereditary sporadic pathologies. The debut occurs during the first year of life. With this disease, there are:

isolated lesion of an erythrocyte germ;
pathological changes in bones;
characteristic eye damage;
the skin acquires a characteristic grayish tint;
hepatosplenomegaly appears early;
platelets and leukocytes are contained in the blood in normal quantities, a decrease is noted only in case of significant damage to the spleen;
characteristic phenotype: enlarged upper lip, widely spaced eyes, pallor of the skin, dry hair and bone age lagging behind the passport one;
this severe disease is fatal before the age of 20. In laboratory studies - a picture of normochromic anemia with normal proliferation of platelet and granulocytic germs;
An effective treatment tactic for this form of anemia is therapy with glucocorticosteroid hormones in combination with transfusion of erythrocyte mass according to vital indications.

Acquired Forms

Depending on the predominant lesion of one or another of the germs, it is advisable to divide all the manifestations observed in aplastic anemia into the main clinical syndromes.

Actually anemia:

dizziness of varying severity;
physical weakness;
noise in ears;
respiratory failure, shortness of breath;
feeling of increased heartbeat.

Hemorrhages:

bleeding, loose gums;
the appearance of hematomas not caused by trauma;
nosebleeds;
there is a possibility of hemorrhage in the brain.

Granulocytopenia:

decrease in the protective function of the body's immune system, frequent incidence of various infections;
purulent inflammation of soft tissues, even with minor injuries;
the occurrence of abscesses at the injection site;
and bruising on the skin;
punctate rashes on the skin;
characteristic murmur on auscultation of the heart;
heart rate is increased;
possible complication in the form;
in severe cases, hepatomegaly is detected, and scleral icterus may be noted.

The course of the disease. Severity

According to the results of a blood test and bone marrow trephine biopsy, various degrees of severity of aplastic anemia are distinguished.

Aplastic anemia of moderate severity:

bone marrow with trepanobiopsy is hypotrophic;
the content of granulocytes is not more than 2 * 100 / l;
platelets - up to 100 * 100 / l;
reticulocytes less than 2-3%.

Severe aplastic anemia:

bone marrow is aplastic on trepanobiopsy;
granulocytopenia less than 0.5*100/l;
less than 20*100/l;
reticulocytes - up to 1%.

Extremely severe degree of aplastic anemia:

granulocytes less than 0.2*100/l;
platelets are single or not found;
reticulocytes - single or absent.

Diagnostics

Assume the diagnosis of aplastic anemia allow the characteristic clinical picture of the disease and the patient's complaints.

The most informative clinical studies:

General blood analysis. Pancytopenia is typical for this pathology, while leukocytopenia appears mainly due to a decrease in the number of granulocytes. In addition, KLA may reflect signs of an inflammatory process.
Picture of biochemical indicators of blood. In aplastic anemia, the following parameters may be subject to characteristic changes: thymol test, C-reactive protein, HBs antigen, erythropoietin, and serum iron levels.
Sternal puncture allows visualization of bone marrow cells to assess their quantitative ratio and the degree of inhibition of hematopoiesis. Myelogram (the so-called result of the study) reflects the cellular composition of the bone marrow.
Trepanobiopsy involves taking a fragment of bone marrow from the ilium for analysis. The advantage over sternal puncture is that more material is taken and that its structural integrity can be preserved.

A complete blood count and the result of a trepanobiopsy allow us to assess the severity of aplastic anemia and determine the form of the disease.

Treatment of aplastic anemia

In cases where the etiopathogenetic factor that caused the disease is known, therapy will begin with attempts to eliminate the causes that provoked the development of the disease. Such measures are designed to eliminate the likelihood of further progression of the pathological process.

Each form of aplastic anemia corresponds to a specific treatment regimen, depending on the characteristics of the course of the pathological process.

With clinically confirmed aplastic anemia, treatment can be carried out in three main directions.

replacement therapy. The method involves transfusion of blood - whole, formed elements (platelet or erythrocyte mass, granulocytes) or blood substitutes. This is a preventive method of treatment, only temporarily replenishing the required number of blood cells, however, it does not restore the function of hematopoiesis.

It should be borne in mind that this method is not applicable in the autoimmune form of aplastic anemia, since the reaction to the ingestion of a foreign protein will be an immune response against the introduced cells.

In such a situation, replacement therapy will be ineffective.

It is advisable to combine replacement therapy with the use of agents that promote the removal of iron from the body, since repeated blood transfusions lead to the accumulation of this element in the body.

Bone marrow transplant. This method of treatment of aplastic anemia is considered to be the most effective today. The use of the method is preferable at a young age. For the success of treatment, it is necessary to choose the right donor. They may be a close relative of the patient.

And also properly prepare the patient (such preparation involves chemotherapy and exposure to large doses of radiation). The operation involves injecting filtered healthy stem cells from a donor into the recipient's bloodstream. Through the blood flow, the donor material enters the bone marrow of the patient, where, subsequently, the proliferation of healthy blood cells occurs.

To prevent rejection of the transplanted material, the patient is given appropriate treatment that suppresses the immune response.

Medical therapy:

designed to stimulate the processes of hematopoiesis;
immunosuppressive - relevant for the autoimmune form of aplastic anemia. Such therapy prevents an inadequate immune response.

Prognosis for aplastic anemia

In severe aplastic anemia, despite the development of the pharmaceutical industry, there is still a high mortality rate.

Factors that improve prognosis:

the development of transplantology and the beginning of the use of transplantation;
immunosuppressive therapy.