Disease of the hematopoietic system. Oncogenetic syndromes of the hematopoietic and immune systems
Department of propaedeuticsinternal medicine
anemia
Iron deficiency anemia (IDA)
Iron deficiency is one of the most
widespread deficits in the world ($2 billion)
people), is considered the "culprit" of 0.8 million.
(1.5%) of deaths worldwide.
In the world of IDA, 18% of men and 35%
women (mainly in developing
countries). In Russia, IDA occurs in 13.5%
in women of childbearing age.
Vitamin B12 deficiency anemia
The most common diseases of the blood system
Hemorrhagic diathesis- conditions with increased bleeding
Violations vascular wall
Platelet pathology
Coagulation disorders
Hemoblastoses
(myeloproliferative and lymphoproliferative
disease) - malignant diseases systems
blood; account for 1.5 - 2.6% among therapeutic
diseases.
Leukemia is a tumor primary lesion bone
brain (acute and chronic leukemias)
Lymphomas - with tumor growth outside the bone marrow
(lymphoid tissue, other organs)
Main complaints
General weakness, dizziness, fainting, shortness of breath andpalpitations during exercise (combined with
pallor of the skin and mucous membranes) - circulatory-hypoxic syndrome with anemia
Fever
Prolonged subfebrile condition - often with anemia
(hemolytic, vitamin B12-deficient, etc.)
High fever with chills, profuse sweat, weight loss
- with leukemia, lymphomas, lymphogranulomatosis
(wavy character)
Fever infectious complications in patients with acute
leukemia, agranulocytosis
Bleeding (hemorrhagic rashes on the skin and
mucous membranes, nasal, gastrointestinal, uterine
bleeding) with hemorrhagic syndrome
Weight loss, loss of appetite - with malignant tumors
blood systems (leukemias, lymphomas)
Itching of the skin with lymphogranulomatosis, chronic
lymphocytic leukemia, erythremia
Main complaints
Perversion of smell and taste (pica chlorotica)iron deficiency anemia
Burning tongue - Gunther's glossitis with vitamin B12 deficiency anemia
Sore throat due to necrotizing sore throat
with agranulocytosis, acute leukemia
Bone pain due to bone marrow hyperplasia
leukemia, multiple myeloma, cancer metastases
Pain and heaviness in the left hypochondrium with significant
enlargement of the spleen, perisplenitis, infarction and rupture
spleen
Pain and heaviness in the right hypochondrium: with
significant enlargement of the liver in leukemia and
lymphomas; with hemolytic anemia by type
hepatic colic (due to the formation of bilirubin
stones)
Anamnesis of life
Nutrition - deficiency of iron, folic acid, vitaminsgroups B and K
Occupational history: lead poisoning -
anemia, benzene - aplastic anemia and leukemia;
radiation - leukemia, etc.
Severe diseases of the internal organs
Diseases of the gastrointestinal tract - iron, vitamin
B12 deficiency anemia
Liver disease – clotting disorders, anemia
Kidney disease - renal anemia
Rheumatic diseases, chronic infections - anemia
chronic diseases
Hypoxia in pulmonary heart failure -
secondary erythrocytosis; bleeding during
bronchiectasis and tuberculosis - anemia
Injuries and operations (gastrectomy - malabsorption
iron and vitamin B12; splenectomy - secondary
thrombocytosis; extraction of teeth - reveals violations
hemostasis)
Anamnesis of life
Medications (analgin, NSAIDs, sulfonamides -agranulocytosis; β-lactams - hemolytic anemia;
heparin, biseptol - thrombocytopenia)
Alcohol - clotting disorders, anemia,
thrombocytopenia (toxic effect of alcohol on bone
brain and liver)
Smoking increases the risk of cancer
various blood disorders
Allergies - the cause of eosinophilia
Blood transfusions
Heredity - hemophilia, hereditary
hemolytic anemia and other hematological diseases
Ethnicity - sickle cell
anemia, thalassemia, etc.
Gynecological status - reveals problems of hemostasis,
possible causes of anemia
General inspection
General stateConsciousness - anemic coma
Skin examination
Pale skin
Alabaster pallor in iron deficiency anemia
Waxy skin with vitamin B12 deficiency anemia
Paleness with an earthy gray tint
Icteric coloration in hemolytic anemia (pale yellow skin)
Red-cherry color with erythremia (plethora)
Skin hemorrhages (petechiae, ecchymosis, purpura)
scratch marks due to itching
Leukemids (for acute myeloblastic leukemia)
Dryness and flaking of the skin, brittle hair, changes
nails with iron deficiency (koilonychia)
Skin pallor due to vitamin B12 deficiency anemia
Pallor of the nail bed
cachexia, pallor
Jaundice
erythremia
Hemorrhagic rash
Koilonychia in iron deficiency
Inspection of body parts
Examination of the oral cavity and tongueAngular stomatitis - "jams", cracks in the corners of the mouth (with
deficiency of iron and B vitamins)
Tooth decay and inflammation of the mucosa around the necks
teeth (alveolar pyorrhea) with iron deficiency anemia
Gunther's glossit ("varnished" crimson tongue, With
papillary atrophy) in B12 deficiency anemia
Ulcerative necrotic angina and stomatitis in acute
leukemia
Gingival hyperplasia in acute monoblastic leukemia
Inspection of the neck, over- and subclavian areas, axillary
hollows, etc.
- an increase in lymph nodes - local or generalized
Examination of the abdomen
- bulging of the left side of the abdomen with splenomegaly
Seizures in the corners of the mouth with a deficiency of iron and B vitamins
"Hunter's language" in B12-deficiency anemia
Gingival hyperplasia in acute monoblastic leukemia
Bleeding gums, lips
Telangiectasias in the mouth and tongue
Palpation of lymph nodes
The main reasons for the increase in lymph nodes:Blood diseases: chronic lymphocytic leukemia,
Hodgkin's disease, lymphomas, acute
lymphoblastic leukemia
Infections: HIV, cytomegalovirus, rubella,
infectious mononucleosis, toxoplasmosis,
tuberculosis, chlamydia, sepsis
Diffuse connective tissue diseases:
Systemic lupus erythematosus, juvenile
rheumatoid arthritis
Local infections with regional lymphadenitis
Cancer metastases
Palpation of the lymph nodes
Characteristics of palpable lymph nodesnodes:
Localization
Size
Consistency
Displacement
Soreness
Skin changes over lymph nodes
Enlarged cervical lymph nodes
Enlargement of lymph nodes
Metastases of gastric cancer in the left supraclavicular node
Palpation of the spleen
Splenomegaly - enlargement of the spleenBlood diseases
Myeloproliferative diseases (chronic
myeloid leukemia, idiopathic myelofibrosis, etc.)
Lymphoproliferative diseases (lymphomas,
hron. lymphocytic leukemia, acute lymphoblastic leukemia)
Hemolytic anemia
Other reasons
Infections (sepsis, tuberculosis, toxoplasmosis, malaria,
syphilis, HIV, viral hepatitis and etc.)
Diffuse connective tissue diseases
Portal hypertension (liver cirrhosis)
Thrombosis of the splenic vein, stagnation of blood in the spleen
with heart failure
Storage disease, spleen amyloidosis
Splenomegaly. Hepatomegaly.
Palpation of the liver in blood diseases:
Hepatomegaly in blood diseases:Acute leukemia
Chronic myeloid leukemia
Chronic lymphocytic leukemia
Lymphomas
Percussion
Dimensions of the liver and spleenIncrease in mediastinal
lymph nodes - dullness in
interscapular space
Tapping on the bones
Auscultation
Auscultation of the heart in anemia:tachycardia
amplification of the volume of the I tone (NB: when
chronic anemia - weakening of the I tone for
account of myocardial dystrophy)
functional systolic murmur
top
the noise of the "top" on the jugular vein
Rubbing noise of the peritoneum during perisplenitis
Laboratory research methods
General clinical blood testPuncture of the bone marrow
Trepanobiopsy
Puncture and surgical biopsy
lymph node
Puncture of the spleen
Immunophenotyping (definition
membrane antigens - CD antigens)
Cytogenetic study
General blood analysis
IndicatorsNormal values
Hemoglobin, g/l
Men 140 - 160; women 120 – 140
Erythrocytes, x 1012/l
Men 4 - 5.1; women 3.7 – 4.7
Color indicator
0,82 – 1,05
Reticulocytes, %0
2 – 12
Platelets, x 109/l
180 - 320
Leukocytes, x 109/l
4 – 8,8
Young neutrophils, %
0–1
Stab, %
1–6
Segmentonuclear, %
45 – 70
Basophils, %
0–1
Eosinophils, %
0–5
Lymphocytes, %
18 – 40
Monocytes, %
2–9
ESR, mm/h
Men 1 - 10; women 2 – 15
Erythrocyte indices
MCV (mean corpuscular volume) - the average volume of an erythrocyteHematocrit (l/l) x 1000
Normal values: 80 – 95 µm3 (fl)
MCH (mean corpuscular hemoglobin)
hemoglobin in erythrocyte
Hemoglobin (g/l)
Number of erythrocytes (х1012/l)
Normal values 27 – 34 pg/cell
MCHC (mean corpuscular hemoglobin concentration) - average
concentration of hemoglobin in the erythrocyte
Hemoglobin (g/l)
Hematocrit (l/l)
Normal values 31 – 36 g/dl RBC concentrate
Biochemical research methods
Bilirubin metabolism - withhemolytic anemia
Whey content
iron, transferrin, ferritin
Other
X-ray examination, CT, ultrasound
Enlargement of mediastinal lymph nodes– Ro-graphy and chest CT
Increase in intra-abdominal
lymph nodes, liver, spleen - ultrasound
and CT scan of the abdomen
X-ray of flat bones
myeloma
X-ray of the skull in multiple myeloma
Anemia syndrome
Anemia is a condition characterized bya decrease in hemoglobin (and
erythrocytes) per unit volume of blood
Anemia is indicated by a decrease
hemoglobin below 120 g/l and hematocrit
below 37% in women and, accordingly,
below 140 g/l and 40% in men
Causes of anemia
Erythropoiesis disorderI.
Violation of hemoglobin synthesis -
Iron-deficiency anemia
Violation of the synthesis of nucleic acids -
vitamin B12/folate deficiency anemia
Damage to the erythropoietic stem
cells - aplastic anemia,
bone marrow metaplasia in acute
leukemia, with cancer metastases to the bone
brain
Erythropoietin deficiency - anemia
kidney failure
Causes of anemia
Increased destruction of red blood cells(hemolytic anemia)
II.
Congenital - hereditary microspherocytosis And
others
Acquired - autoimmune and isoimmune
hemolytic anemia, hemolytic disease
newborns, mechanical damage
erythrocytes (eg, with artificial valves),
infections (malaria), poisoning with hemolytic
poisons, etc.
Blood loss -
III.
Acute posthemorrhagic anemia
Chronic post-hemorrhagic anemia -
Iron-deficiency anemia
Classification of anemia by erythrocyte morphology
Microcytic hypochromic - iron deficientanemia
Normocytic normochromic - autoimmune
hemolytic anemia, aplastic anemia
Macro-(megalo)cytic hyperchromic - deficiency
vitamin B12/folic acid
Anisocytosis - the appearance of red blood cells of different diameters
Poikilocytosis - the appearance of red blood cells different shapes
Classification of anemia according to the degree of regeneration
Depending on the number of blood reticulocytesand erythrokaryoblasts of the bone marrow are distinguished:
(Hyper)regenerative anemias - increased
the number of blood reticulocytes (and bone marrow erythrokaryoblasts) - hemolytic
anemia, acute posthemorrhagic anemia
Hypo- and regenerator anemias - the absence
increase in blood reticulocytes (and bone marrow erythrokaryoblasts) - aplastic
anemia, iron and vitamin B12 deficiency
anemia before treatment.
Classification of anemia by severity
SeverityI degree (light)
II degree (medium)
III degree (severe)
IV degree (extreme
severity)
Content
hemoglobin
140 - 100 g/l (men)
120 - 100 g/l (women)
99 – 67 g/l
66 – 34 g/l
< 33 г/л
Complaints:
weakness, fatigue
dizziness, tinnitus, tendency to
fainting
shortness of breath (especially with exertion)
palpitations (especially during exercise)
There may be dyspeptic complaints:
taste perversion (with iron deficiency)
anemia), loss of appetite, nausea,
fast saturation
Anemia Syndrome: Clinical Manifestations
Inspection:Paleness of the skin and mucous membranes
Trophic changes in the skin and its appendages (with
iron deficiency anemia)
Dry skin
Brittleness, hair loss, early graying
Fragility, transverse striation of nails, koilonychia
Oral cavity:
Cracks in the corners of the mouth (angular stomatitis)
Smoothing of the papillae of the tongue (atrophic glossitis with
iron deficiency)
"Hunter's language" in B12-deficiency anemia
Teeth lose their shine, decay quickly, maybe
periodontal disease, alveolar pyorrhea (with iron deficiency)
Anemia Syndrome: Clinical Manifestations
Percussion of the heart:Auscultation of the heart:
Heart sounds are accelerated, the I tone is strengthened (but: at
chronic anemia due to myocardial dystrophy
weakened), functional systolic murmur on
apex of the heart, "top noise" on the jugular vein
Palpation of the pulse:
Expanding the boundaries of relative cardiac dullness
to the left due to myocardial dystrophy in chronic
anemia
The pulse is rapid, weak filling (filamentous)
Trend to lower blood pressure
Laboratory data: decrease in hemoglobin,
erythrocytes, ESR acceleration
Normal blood smear
Blood smear for vitamin B12 deficiency anemia
Blood smear for iron deficiency anemia (hypochromia and microcytosis of erythrocytes)
Blood smear for iron deficiency anemia (hypochromiaand microcytosis of erythrocytes)
Hemorrhagic syndrome Impaired hemostasis
I. Pathology of platelets -Decreased platelet count
(thrombocytopenia)
e.g. idiopathic thrombocytopenic
purpura (Werlhof's disease),
thrombocytopenia in leukemia, lymphoma and
cancer metastases to the bone marrow, with vitamin
B12 deficiency anemia; with liver diseases
(hepatolienal syndrome), etc.
Platelet dysfunction
(thrombocytopathy)
Hemostasis disorders
II. Pathology of blood coagulation -coagulopathy
Secondary coagulopathy in diseases
liver, vitamin K deficiency and
other diseases
Primary (congenital) coagulopathy:
hemophilia A, B
Violations
III. Vascular pathologyVasculitis
Hemorrhagic vasculitis of Schonlein of Henoch
Vasculopathy
hereditary telangiectasia
(Rendu-Osler disease)
Hemorrhagic syndrome
Complaints:Appearance on the skin and mucous membranes
hemorrhages of various nature
(small spots, bruises, etc.)
Bleeding (nose,
gingival, uterine, gastrointestinal,
renal, etc.) and hemorrhages in
various organs (brain,
retina, joints)
Objective examination. Bleeding types:
Hematoma - with large tensecollections of blood escaping from blood vessels
infiltrating subcutaneous tissue, muscles, etc.
soft tissues– typical of factor deficiency
coagulation (eg, hemophilia A and B). Also for
hemophilia is characterized by bleeding into the joints
(hemarthrosis)
Petechial-spotted (bluish) -
thrombocytopenia, thrombocytopathy Hemorrhagic vasculitis
Hereditary telangiectasia (Rendu-Osler disease)
Proliferative syndrome in hemoblastoses
Pathological cell proliferation of one of the germshematopoiesis (myeloid, lymphoid,
erythrocyte, etc.)
Decrease (with chronic leukemia) or complete absence
(in acute leukemia) cell differentiation, which
leads to the entry into the blood of immature cells of this
proliferating germ
Bone marrow metaplasia with
exclusion of other hematopoietic sprouts
(erythrocyte, platelet)
Development in various bodies leukemoid
infiltrates - pathological growths of cells
proliferating hematopoietic lineage,
metastasized to these organs
The main clinical signs of leukemia
proliferative syndromeHyperplasia of the hematopoietic tissue (increased lymph nodes,
spleen, liver) and the occurrence of foci
extramedullary hematopoiesis (leukemids - skin
infiltrates, ossalgia, pain when tapping
bones)
Anemic syndrome - due to bone marrow metaplasia and
inhibition of the erythrocyte germ
Hemorrhagic syndrome - due to bone marrow metaplasia
and inhibition of megakaryocytic germ and thrombocytopenia
Decreased immunological resistance (infectious-septic and ulcerative-necrotic processes in the lungs,
kidneys, tonsils, etc.)
Corresponding changes in blood and bone marrow tests
Changes in the leukocyte formula
myeloblastPromiel
ocyte
Myelocyte
Metamyelocyte
rod nuclear
neutrophil
0–1%
1–6%
Segmented
neutrophil
45 – 70%
Jet shift left
Hypersegme
ntirovann
th neutrophil
(0 – 1%)
Norm
infections
Pathological shift to the left
Chronic
myeloid leukemia
Leukemic failure
(Hiatus leucaemicus)
Degenerative shift to the right
Spicy
leukemia
B12-deficient
anemia
Diseases of the blood system are divided into anemia, leukemia and diseases associated with damage to the hemostasis system (blood clotting).
Causes that cause damage to the blood system.
anemia.
Among the most common causes that cause anemia, are important:
- acute blood loss (trauma);
- chronic blood loss different localization(gastrointestinal, uterine, nasal, renal) due to various diseases;
- malabsorption in the intestine of iron, which comes with food (enteritis, intestinal resection);
- increased need for iron (pregnancy, feeding, rapid growth);
- common dietary iron deficiency (malnutrition, anorexia, vegetarianism);
- vitamin B12 deficiency (insufficient intake of it with food is meat and dairy products, malabsorption of this vitamin: with atrophic gastritis, after gastric resection, due to hereditary factors, at toxic effects alcohol, with diseases of the pancreas, with invasion with a wide tapeworm);
- malabsorption of folic acid; bone marrow diseases; various hereditary causes.
Leukemias.
The reasons are not fully understood, but the following is known that it may be a hereditary predisposition, ionizing radiation, chemical substances(varnishes, paints, pesticides, benzene), viruses. The defeat of the hemostasis system is most often due to hereditary factors.
Symptoms of blood diseases.
Often patients with blood diseases complain of weakness, easy fatigue, dizziness, shortness of breath when physical activity, interruptions in the work of the heart, loss of appetite, decreased efficiency. These complaints are usually manifestations various anemias. With acute and profuse bleeding suddenly there is a sharp weakness, dizziness, fainting.
Many diseases of the blood system are accompanied by fever. low temperature observed in anemia, moderate and high occurs in acute and chronic leukemia.
Also, patients often complain of itching of the skin.
In many diseases of the blood system, patients complain of loss of appetite and weight loss, usually especially pronounced, turning into cachexia.
For B12-deficiency anemia, patients feel a burning sensation at the tip of the tongue and its edges, with iron deficiency anemia, a perversion of taste is characteristic (patients willingly eat chalk, clay, earth, coal), as well as smell (patients experience pleasure from inhaling ether, gasoline and other vapors). odorous substances with bad smell).
Also, patients may complain of various skin rashes, bleeding from the nose, gums, gastrointestinal tract, lungs (with hemorrhagic diathesis).
There may also be pain in the bones when pressed or tapped (leukemia). Often in blood diseases pathological process the spleen is involved, then there are severe pain in the left hypochondrium, and with the involvement of the liver - in the right hypochondrium.
There may be enlarged and painful lymph nodes, tonsils.
All of the above symptoms are a reason to see a doctor for an examination.
During the examination, the patient's condition is determined. Extremely severe can be observed with final stages many blood diseases: progressive anemia, leukemia. Also, on examination, pallor of the skin and visible mucous membranes is revealed, with iron deficiency anemia, the skin has “alabaster pallor”, with B12 deficiency it is slightly yellowish, with hemolytic anemia it is icteric, with chronic leukemia the skin has an earthy gray tint, with erythremia - cherry red. With hemorrhagic diathesis, hemorrhages are visible on the skin and mucous membranes. The state of trophism also changes skin. With iron deficiency anemia, the skin becomes dry, flaky, hair becomes brittle, split ends.
When examining the oral cavity, atrophy of the papillae of the tongue is revealed, the surface of the tongue becomes smooth (B12-deficiency anemia), rapidly progressive tooth decay and inflammation of the mucous membrane around the teeth (iron deficiency anemia), ulcerative necrotic tonsillitis and stomatitis (acute leukemia).
Palpation reveals soreness of flat bones (leukemia), enlarged and painful lymph nodes (leukemia), an enlarged spleen (hemolytic anemia, acute and chronic leukemia). With percussion, you can also detect an enlarged spleen, and with auscultation, the noise of friction of the peritoneum over the spleen.
Laboratory and instrumental research methods.
Morphological examination of blood: general blood analysis(determination of the number of erythrocytes and the content of hemoglobin in them, determination total number leukocytes and ratio individual forms among them, determining the number of platelets, erythrocyte sedimentation rate). With iron deficiency anemia, the level of hemoglobin and the number of erythrocytes unevenly decrease, hemoglobin decreases more strongly. With B12-deficiency anemia, on the contrary, the number of red blood cells is reduced more than hemoglobin, and with this form of anemia, increased red blood cells can be detected. Changes in leukocytes (qualitative and quantitative composition) is observed in leukemia.
Morphological evaluation of erythrocytes reveals anemia.
Puncture hematopoietic organs . The morphological composition of blood is not always sufficient reflects changes occurring in the hematopoietic organs. So, in some forms of leukemia, the cellular composition of the blood is almost not disturbed, despite significant changes in the bone marrow. For this, a sternal puncture is used (they take bone marrow from the sternum). Bone marrow puncture allows you to identify violations of cell maturation - an increase in the number of young forms or the predominance of primary undifferentiated elements, violations of the ratio between the cells of the red (erythrocyte) and white (leukocyte) series, changes in the total number of blood cells, the appearance of pathological forms and much more. In addition to the sternum, bone marrow can also be extracted from other bones, such as the ilium.
More accurate information about the composition of the bone marrow gives trephine biopsy when the ilium column is cut out together with the bone marrow tissue, and from which histological preparations are made. They preserve the structure of the bone marrow, and the absence of blood impurities allows you to more accurately assess it.
Enlarged lymph nodes are often punctured, and the nature of changes in the cellular composition can be assessed lymph nodes and clarify the diagnosis of diseases of the lymphatic apparatus: lymphocytic leukemia, lymphogranulomatosis, lymphosarcomatosis, detect tumor metastases and others. More accurate information can be obtained with a biopsy of the lymph node, puncture of the spleen.
A comprehensive study of the cellular composition of the bone marrow, spleen and lymph nodes allows us to clarify the nature of the relationship between these departments. hematopoietic system, to reveal the presence of extramedullary hematopoiesis in some lesions of the bone marrow.
Assessment of hemolysis necessary in identifying the hemolytic nature of anemia (free bilirubin is determined, a change in the osmotic stability of erythrocytes, the appearance of reticulocytosis).
Study of hemorrhagic syndrome. Classical coagulation tests are distinguished (determination of blood clotting time, platelet count, bleeding duration, retraction blood clot, capillary permeability) and differential tests. The clotting time characterizes the coagulability of blood as a whole and does not reflect the individual phases of coagulation. The duration of bleeding is determined by the Duke prick test, normally 2 to 4 minutes. Capillary permeability is determined using the following tests: tourniquet symptom (the norm is more than 3 minutes), jar test, pinch symptom, hammer syndrome and others. Differential tests: determination of plasma recalcification time, prothrombin consumption test, determination of the prothrombin index, plasma tolerance to heparin, and others. The summarized results of the listed samples constitute a coagulogram characterizing the state of the blood coagulation system. X-ray examination, it is possible to determine an increase in the lymph nodes of the mediastinum (lympholeukemia, lymphogranulomatosis, lymphosarcoma), as well as changes in the bones that can be in some forms of leukemia and malignant lymphomas (focal destruction of bone tissue in multiple myeloma, bone destruction in lymphosarcoma, bone compaction in osteomyelosclerosis ).
Radioisotope research methods allow to evaluate the function of the spleen, determine its size and identify focal lesions.
Prevention of blood diseases
Prevention of diseases of the blood system is as follows, it is timely diagnosis and treatment of diseases that are accompanied by blood loss (hemorrhoids, peptic ulcer, erosive gastritis, nonspecific ulcerative colitis, uterine fibromatosis, hernia esophageal opening diaphragm, bowel tumors), helminthic invasions, viral infection, if it is impossible to recover from them, then it is recommended to take iron supplements, vitamins (especially B12 and folic acid) and, accordingly, use food products containing them, and these measures should also be applied to blood donors, pregnant and lactating women, patients with profuse menstruation.
Patients with aplastic anemia should take measures to prevent exposure to the body external factors such as ionizing radiation, dyes and others. They also need dispensary observation and control of blood tests.
For the prevention of diseases of the blood coagulation system, family planning (prevention of hemophilia), prevention of hypothermia and stressful situations, vaccinations, tests with a bacterial antigen, alcohol (with hemorrhagic vasculitis), refusal to conduct unreasonable blood transfusions, especially from different donors, are contraindicated.
For the prevention of leukemia, it is necessary to reduce, if any, exposure harmful factors such as ionizing and non-ionizing radiation, varnishes, paints, benzene. To prevent serious conditions and complications, you do not need to self-medicate, but consult a doctor if any symptoms appear. If possible, try to pass annually medical examination be sure to take a general blood test.
Diseases of the blood, hematopoietic organs and certain disorders involving the immune mechanism according to ICD-10
Diet related anemia
Anemia due to enzyme disorders
Aplastic and other anemias
Blood clotting disorders, purpura and other hemorrhagic conditions
Other diseases of the blood and blood-forming organs
Individual violations that involve the immune mechanism
Anemia is a polyetiological disease characterized by changes in external signs(pallor of the skin, mucous membranes, sclera, often masked by jaundice), the appearance of disorders from muscular system(weakness, decrease in tissue turgor), deviations in the central nervous system (lethargy, apathy, mild excitability), functional disorders cardiovascular system (tachycardia, expansion of boundaries, appearance systolic murmur in t. Botkin and the apex of the heart), the development of hepato- and splenomegaly, a change in the morphology of erythrocytes (a decrease in volume, a change in shape, osmotic stability), a change in the content of other cellular forms (leukocytes, platelets) of bone marrow punctate, electrolyte metabolism and the content of iron and magnesium in blood serum.
The classification is as follows.
1. Deficiency anemia: iron deficiency, vitamin deficiency, protein deficiency.
2. Hypo- and aplastic anemia: Fanko-ni congenital anemia, Dabiond-Biekfen anemia, acquired anemia.
3. Hemolytic anemia: spherocytic, sickle cell, autoimmune.
By severity:
1) mild anemia: hemoglobin within 90 - 110 g / l, the number of red blood cells decreases to 3 minutes;
2) anemia of moderate severity: hemoglobin 70–80 g/l, erythrocytes up to 2.5 minutes;
3) severe anemia: hemoglobin below 70 g/l, erythrocytes below 2.5 min. By functional state erythropoiesis:
a) regenerative anemia: retiulocytes more than 50%;
b) hypo- and regenerator anemia: low retilunocytosis, inadequate severity of anemia. Downstream: acute phase, subacute and chronic course.
Iron-deficiency anemia
Iron deficiency anemia is a disease that is caused by iron deficiency in the blood serum, bone marrow and depot, which leads to the development of trophic disorders in tissues. The development of anemia is preceded by latent tissue iron deficiency. It is more common in women than in men, in 14% of women of childbearing age living in the middle lane.
Etiology: the causes of iron deficiency anemia are chronic blood loss, insufficient initial iron levels, which manifests itself during puberty; malabsorption and intake of iron with food. Most often, several unfavorable factors are combined. Agistral and entologenic anemias are often accompanied by a deficiency of not only iron, but also vitamin B 12, folic acid, and proteins.
Classification:
1) chronic posthemorrhagic;
2) due to hemoglobinuria and hemosiderinemia;
3) iron deficiency in donors (withdrawal of 400-500 ml of blood is accompanied by a loss of 200-250 mg of iron.).
Clinic. In anamnesis, insufficient, improper, unilateral nutrition, frequent diseases. Dryness, roughness of the skin, fragility of hair, pallor of mucous membranes, atrophy of the papillae of the tongue; functional changes gastrointestinal tract, leading to spasms of the esophagus, accelerated intestinal peristalsis, splenitis and hepatomegaly.
Changes in the morphology of erythrocytes and biochemical parameters of blood serum, anisocytosis, poikilocytosis, a decrease in the osmotic ability of erythrocytes, a decrease in the concentration of serum iron, an increase in the content of copper in the blood serum.
In the mechanism of development of clinical manifestations of iron deficiency anemia, tissue hypoxia and a decrease in the activity of most enzymes are of paramount importance.
Patients have severe weakness, darkening before the eyes when the body position changes, headaches, dizziness, fainting, shortness of breath, palpitations during slight physical exertion, increased tooth decay, smoothness of the papillae of the tongue. In severe cases - violation, discomfort when swallowing (Bekhterev's symptoms) of dry and solid food, raspberry color of the tongue, atrophic changes in the pharynx and esophagus, spastic narrowing of the upper esophagus, fragility, the formation of longitudinal or transverse striation along the nail plate, koilonychia. Perversion of taste sensations (addiction to honey, tooth powder, chalk, dry cereals, coal, lime, ice, smells of gasoline, kerosene) indicates a violation of peripheral taste sensitivity. Patients may complain of muscle weakness, imperative urge to urinate, enuresis. Hyporegeneration of blood cells is caused by a decrease in the proliferative capacity of the bone marrow and ineffective hematopoiesis. Differential diagnosis is carried out with thalassemia, posthemorrhagic and infectious anemia.
Treatment
The principles of treatment are as follows.
1. Active mode.
2. Balanced diet.
3. Iron preparations in combination with ascorbic acid and copper.
4. Aerotherapy, massage, gymnastics.
5. Food enzymes.
6. Blood transfusion when the hemoglobin content is less than 60 g / l, iron preparations are administered orally between meals, since during this interval there is a better absorption. With iron intolerance (decreased appetite, nausea, vomiting, epigastric pain, dyspepsia, allergic dermatoses) in order to avoid damage to the mucosa of the gastrointestinal tract, iron preparations are administered parenterally. Iron preparations include hemostimulin, ferroplex, sorbifer durulez, ferrum lek. Prevention of anemia should be carried out by donors, women with heavy menstruation, frequent pregnancies, girls during puberty, with frequently recurring bleeding.
vitamin deficiency anemia
Pernicious anemia (Addison-Birmer's disease) is caused by vitamin B12 deficiency, which manifests itself in damage to the hematopoietic, digestive and nervous systems. It occurs more often in older age, with the same frequency in men and women.
Etiology. Vitamin deficiency is rarely exogenous, more often endogenous, associated with their increased consumption (helminthiasis) and impaired absorption different nature(stomach disease, malabsorption syndrome). Violation of the absorption of vitamin B 12 is most often caused by atrophy of the gastric mucosa and the absence or decrease in secretion internal factor, hydrochloric acid, pepsin. There is a hereditary predisposition associated with a violation of the secretion of the internal factor; violation of immune mechanisms (detection of antibodies to own cells). Pernicious anemia may occur after gastrectomy, resection.
Clinic
In patients with pernicious anemia, the skin acquires a lemon-yellow hue, the formation of mottled brown pigmentation is possible. Patients complain of weight loss caused by anorexia, possibly an increase in body temperature. Half of the patients have symptoms of glossitis, sometimes the mucous membranes of the cheeks, gums, pharynx, esophagus are affected, diarrhea develops, the liver enlarges, the spleen often enlarges - hepatosplenomegaly; shortness of breath, palpitations, extrasystole, weakness, dizziness, tinnitus. Characteristic of pernicious anemia is a rheumatic syndrome, which is caused by a lesion white matter spinal cord. In severe cases, there are signs of damage to the posterior columns of the spinal cord, unsteady gait, impaired coordination of movements, ataxia, hyperreflexia, tone of the feet. Rare, but dangerous symptoms are mental disorders, maximum outbreaks, paranoid states.
Differential Diagnosis performed with familial megaloblastic anemia.
Survey plan.
2. Examination of feces for occult blood.
3. Blood test for reticulocytes, platelets.
4. Determination of iron content in the blood (if iron deficiency anemia is suspected).
5. Fibrogastroduodenoscopy.
6. Colonoscopy (if irrigoscopy is impossible).
7. X-ray examination of the lungs.
8. Sternal puncture, myelogram study.
9. Biochemical analysis of blood (total protein and protein fractions, bilirubin, transaminases).
10. Study of the secretory function of the stomach.
Principles of treatment.
1. Diet therapy.
2. Appointment of B 12 and folic acid.
3. Enzyme therapy.
4. Anabolic hormones and insulin.
5. Stimulating therapy.
The best results are obtained by treatment with vitamin B 12 (cyanocobolamine). In severe cases, intravenous administration of the drug at 100–200 mcg during the week. The course dose is 1500-3000 mcg. In severe cases and in the presence of antibodies, the appointment of corticosteroids is indicated.
Hypo- and aplastic anemia
A group of diseases of the blood system, the basis of which is a decrease in the production of bone marrow cells, more often three-cell lines: erythrocyto-, leuko- and thrombocytopoiesis.
Diagnostic criteria. Congenital Fanconi anemia: in the analysis, a gradual increase in pallor of the skin and mucous membranes, weakness, lethargy, combined with a lag in physical development, strabismus, hyperreflexia.
In early childhood, melanin brown pigmentation of the skin appears, anomalies of bones, forearms, thumbs, spinal deformity, dwarfism, combined with malformations of the heart, kidneys (heart, kidney failure), sensory organs, central nervous system(microcephaly); changes in the blood: a drop in hemoglobin, pancytopenia, a decrease in reticulocytes, a deficiency of glucose-6-phosphate dehydrogenase, a decrease in the activity of alkaline phosphatase, polysaccharides.
Congenital Estren-Dameshek anemia. In history: manifested at an early stage of development of children with the gradual development of pallor, irritability, apathy; unique appearance: blonde hair, snub nose, widely spaced eyes, thickening of the upper lip with a bright red border; slowing down the rate of ossification in the wrists; pronounced spleno- and hepatomegaly, changes in the blood; increasing iron deficiency anemia; in the bone marrow punctate: pallor of the brain due to the development of hypoplastic erythropoiesis.
Acquired hypo- and aplastic anemia. In history: develop after viral infections, with a predisposition to allergic reactions to drugs and toxins. It is distinguished by the rapid development of clinical symptoms; temperature response(subfebrile condition), pallor, rash on the skin, exanthema on the mucous membranes, stomatitis and tonsillitis, bloody stool, weakness, anorexia, shortness of breath, possible hemorrhages in the brain, adrenal glands:
1) changes in the blood: pancytopenia, a sharp decline hemoglobin, hyperchromia and macrocytosis of erythrocytes, iron content in blood serum is normal or increased;
2) in the bone marrow punctate: fatty degeneration, poverty shaped elements, the absence of young forms of megalocaryocytes.
Survey plan.
1. General analysis of blood, urine.
3. Sterile puncture with myelography.
4. Fibrogastroduodenoscopy, colonoscopy, ultrasound examination of the liver, pancreas, kidneys (to exclude neoplasms).
differential diagnosis. Inhibition of hematopoiesis can occur in osteosclerosis and osteomyelofibrosis. It is also necessary to differentiate aplastic (hypoplastic) anemia from acute leukemia, Werlhof's disease.
Principles of treatment.
1. Transfusion of erythrocyte mass in acute forms.
2. Plasmaphoresis with the introduction of fresh frozen plasma, albumin or rheopolyglucin.
3. Impact on the vascular wall (dicinone, serotonin, rutin, ascorbic acid - ascorutin).
4. Glucocorticoids along with massive doses of antibiotics; B vitamins, folic acid.
5. Aminocaproic acid, anabolic hormones (reta-bolin).
6. Splenectomy.
Hemolytic anemia
These are anemias that develop as a result of the destruction of red blood cells.
Etiology- a group of acquired and hereditary diseases characterized by intracellular or intravascular destruction of red blood cells. Autoimmune hemolytic anemias are associated with the formation of antibodies to the erythrocytes' own antibodies.
Diagnostic criteria: hereditary microspherocytic anemia (Minkowski-Schauffir disease):
1) in history: the first symptoms are detected at any age, they begin as a result of an existing congenital defect in the lipoid structures of the erythrocyte membrane, therefore, it is important to identify relatives suffering from anemia;
2) pallor with a lemon-yellow tint, congenital stegein (tower skull, wide bridge of the nose, high palate, lethargy, weakness, loss of appetite up to anorexia, dizziness);
3) changes in the cardiovascular system, palpitations, shortness of breath, systolic murmur;
4) changes in the gastrointestinal tract: abdominal pain, colic and a significant increase and induration of the liver and spleen;
5) ulcers on the legs - changes in the blood: a drop in the number of erythrocytes to 2.5 minutes, hemoglobin to 70 g / l, an increase in retinulocytes to 30-50%, a decrease in the minimum osmotic resistance of erythrocytes with an increased maximum, an increase in the level of indirect bilirubin, urobillinogen urine, stercobillin in the skin;
6) in the bone marrow punctate - inhibition of the erythroid germ.
sickle cell anemia
Anemia develops at an early age. A family history matters, the identification of abnormal hemoglobin in relatives:
1) pallor or yellowness of the skin, mucous membranes, sclera, asthenia of the body; characteristic appearance: short torso, long thin limbs, narrow shoulders and hips, tower skull, big belly, ulcers on the extremities, hepatosplenomegaly, expansion of the boundaries of the heart, arrhythmia, systolic murmur;
2) changes in the blood: normochromic anemia 2.5-3 minutes, hemoglobin S or its combination with hemoglobin F, anisocytosis, poikilocytosis, target erythrocytes, deficiency of the enzyme glucose-6-phosphate dehydrogenase.
Autoimmune anemia:
1) in history: anemia develops acutely or gradually after viral, bacterial infections, against the background of rheumatism, cirrhosis of the liver, lymphogranulomatosis, etc.;
2) pallor of the skin and mucous membranes, jaundice in 75% of cases, fever, weakness, drowsiness, irritability, headaches, pain in the abdomen, back, splenomegaly, hematuria;
3) changes in the blood: a decrease in the level of hemoglobin, erythrocytes, reticulocytes, bilirubinemia, an increase in the concentration of serum iron; positive Coombs reaction (detection of antibodies to erythrocytes);
4) in urine: hemoglobinuria;
5) in the bone marrow punctate: irritation of the erythrocyte process.
Survey plan.
1. General analysis of blood, urine, feces.
2. Blood test for reticulocytes, platelets.
3. Urinalysis for urobilin and bilirubin.
4. Biochemical analysis for urea, creatinine, transaminases (AIT, AST), bilirubin, total protein and protein fractions.
5. Study of the osmotic stability of erythrocytes.
6. Fibrogastroduodenoscopy.
7. Ultrasonography liver, pancreas and gallbladder.
8. Coulibs reaction (with suspicion of autoimmune anemia).
differential diagnosis. It is carried out with acute leukemia, Werlhof's disease, sepsis, other hemolytic anemias, in which spherocytosis is not detected and the osmotic resistance of erythrocytes increases (thalassemia, etc.).
Principles of treatment.
2. Transfusions of erythrocyte mass, ear blood.
3. Corticosteroids.
4. Glucose 5% with insulin and vitamins B, B 2, B 12, B 6, C.
5. In the absence of effect - immunosuppressants, splenectomy, laser irradiation.
2. Hemorrhagic diathesis
Hemorrhagic diathesis is a group of diseases characterized by impaired hemostasis (vascular, platelet or plasma) and manifested by an increased tendency to bleeding and hemorrhage.
Etiology
The heredity of hemorrhagic conditions is determined by abnormalities of megakaryocytes and platelets, a defect in plasma coagulation factors, and inferiority of the cervical blood vessels.
Acquired hemorrhagic diathesis is caused by DIC, toxic-infectious conditions, liver diseases, and the action of drugs.
Classification
Diets are limited.
I. Disease caused by impaired vascular hemostasis (vasopathy).
1. Shenein's disease - Henoch (simple, rheumatoid, abdurative and fulminant purpura):
1) simple form;
2) chronic form.
2. Hereditary-familial simple purpura (Davis).
3. Anular telangiectatic purpura of Mabocca.
4. Necrotic purpura of Sheldon.
5. Hyperglobulinemic purpura of Waldenström.
6. Hereditary hemorrhagic telangiectasias.
7. Louis-Barr syndrome (capillary telangiectasia of the conjunctiva with ataxia and chronic pneumonia).
8. Kazabakh-Merritt syndrome.
9. Scurvy and Mimer-Barny's disease.
II. Diseases caused by a violation of the platelet mechanism of hemostasis (thrombocytosis, thrombocytopenia):
1) hemorrhagic thrombocytopathy, Werlhof's disease:
a) acute form;
b) chronic form (continuous and recurrent);
2) amegakaryocytic thrombocytopenic purpura (Landolt);
3) autoimmune thrombocytopenia of various origins;
4) thrombocytopenic hymiphragic purpura with acquired autoimmune hemolytic anemia (Fimer-Evans syndrome);
5) thrombocytopenic purpura with chronic purulent tint and exudative diathesis (Ondrich's syndrome);
6) thrombotic thrombocytopenic purpura of Moshkovich;
7) thrombocytopenia in heangiomas (Kazabakh-Merritt syndrome);
8) hereditary properties of thrombopathy:
a) Glanuman's thrombasthenia;
b) Willibrand's thrombopathy;
9) thrombocytopathy in combination with impaired coagulation factors.
III. Diseases caused by a violation of blood clotting factors (quagulopathy):
1) hemophilia A (factor VIII deficiency):
a) hereditary;
b) family;
c) sporadic;
2) hemophilia B (lack of factor IX):
a) hereditary;
b) family;
c) sporadic;
3) hemophilia C (lack of factor XI);
4) pseudohemophilia due to hypoprothrombinemia:
a) idiopathic hypoprothrombinemia;
b) secondary hypoprothrombinemia ( hemorrhagic disease newborns, malabsorption of vitamin K, liver disease, poisoning with chloroform, phosphorus, arsenic);
5) Ouren's pseudohemophelia:
a) congenital form;
b) acquired form;
6) pseudohemophilia due to lack of VII factor:
a) congenital form;
b) acquired form;
7) pseudohemophilia due to lack of fibrinogen (afibrinogenemia):
a) congenital form;
b) acquired form (DIC);
8) pseudohemophilia due to lack of factor X;
9) pseudohemophilia due to lack of fabrinase;
10) pseudohemophilia due to an excess of anticoagulants:
a) idiopathic;
b) immunoallergic;
c) acquired forms.
3. Hemorrhagic vasculitis
Hemorrhagic vasculitis (Shenein-Genoch disease, capillary toxicosis, anaphylactic purpura) is an infectious-toxin-allergic disease, which is based on generalized hyperemic inflammation of the vessels.
Etiology
Cause of acute inflammatory process small joints of the skin, joints digestive tract and kidney has not been fully elucidated.
The degree of activity - I, II, III.
Course: acute, subacute, chronic, recurrent.
Outcome: recovery, transition to a chronic form, outcome in chronic nephritis(A. S. Kalinichenko, 1970).
Diagnostic criteria
Clinical:
1) hemorrhagic skin syndrome: the rash is usually located symmetrically, characterized by stages of the rash, localized on the extensor surfaces of the limbs, around the ankles and knee joints, in the area of the feet, less often in the hips; rashes, as a rule, polymorphic: hemorrhagic papules, erythritic papules, spots; at the beginning of the disease, the rashes are urtical in nature, later they become hemorrhagic, up to necrosis, relapses are characteristic;
2) articular syndrome: joint lesions have a migratory polyatric character with predominant localization in the shin, ankle, elbow, wrist joints, and joint damage is rarely symmetrical;
3) abdominal syndrome: cramping abdominal pain different intensity; pain may be accompanied by intestinal and renal bleeding.
Laboratory research: hematological changes: leukocytosis, neutrophilia, eosinophilia, accelerated ESR, platelet count is sometimes slightly reduced; retraction of the blood clot, the duration of bleeding and the time of blood clotting are not disturbed; hypercoagulability is often detected; urinalysis: in the acute period of the disease, morning proteinuria, hematuria are often detected; in the feces with abdominal syndrome there may be an admixture of blood.
Examination plan:
1) general analysis of blood, urine, feces;
2) study of platelet clotting time;
3) definition of coagulogram;
4) examination of feces for occult blood (Gregersen's reaction).
Differential Diagnosis
It is carried out with thrombocytopathy, thrombocytopenia, co-agulopathy, toxic drug vasculitis, allergic and infectious diseases.
Principles of treatment
1. Hospitalization and bed rest for at least three weeks.
2. Diet with the exception of cocoa, coffee, citrus fruits, strawberries, etc.
3. Heparin therapy.
4. A nicotinic acid in combination with heparin.
5. Prednisolone.
6. Plasmapheresis (with chronic course vasculitis).
4. Thrombocytopathies
Thrombocytopathy is a quantitative and qualitative insufficiency of the platelet link of hemostasis, clinically manifested by hemorrhagic syndromes.
Etiology
Depending on the genesis, two groups are distinguished:
1) thrombocytopenia - a decrease in the number of platelets (Verlhof disease, Wilbrand-Jurgens, Frank, Kazabakh-Merrit disease);
2) thrombocytopathy - a violation of the properties of platelets. In the vast majority of cases, thrombocytopenia is observed, which are based on an immunoallergic conflict.
Classification
By type: primary (idiopathic) and secondary (symptomatic) thrombocytopenia.
Nosological forms: isoimmune, transimmune, heteroimmune, autoimmune.
Werlhof disease
Classification of idiopathic thrombocytopenic purpura (Werlhof's disease)
Course: acute (up to 6 months); chronic: with rare relapses, with frequent relapses, continuously relapsing.
The clinical picture of purpura: dry purpura (skin syndrome); wet purpura (skin syndrome and bleeding).
Immunological tests: positive, negative.
Period: exacerbation, clinical remission, clinical and gamotological remission.
Complications: uterine, gastric, intestinal bleeding, posthemorrhagic encephalopathy, etc.
Diagnostic criteria
Clinical: pale skin and immune membranes:
1) hyperplastic syndrome: enlargement of the spleen, less often - the liver;
2) hemorrhagic syndrome: hemorrhages in the skin, mucous membranes (asymmetrically located, of various shapes and sizes from petechiae to ectomoses, bleeding from various organs(nasal, uterine, intestinal, etc.), positive endothelial tests (symptoms of tourniquet, pinch).
Laboratory Criteria:
1) in general analysis blood decrease in the number of platelets, changes in the morphology of the plates and their functional properties (adhesion, aggregation); violation of blood clot retraction; an increase in the duration of bleeding, a slowdown in blood clotting; decrease in the number of erythrocytes, raticunocytes during the period of bleeding;
2) change in the myelogram: hyperplasia of the megakaryocytic germ with a violation of the functional activity of megapariocytes;
3) immunological: the presence of antiplatelet antibodies. Survey plan.
1. General analysis of blood, urine, feces.
2. Blood clotting time, bleeding time, platelet count.
3. Coagulogram.
4. Feces for occult blood (Gregersen's reaction).
5. Examination of bone marrow punctate (myelogram).
6. Immunological studies for the presence of antiplatelet antibodies.
Differential Diagnosis
It is necessary to distinguish idiopathic thrombocytopenic purpura from allergic anemias, primary liver diseases in combination with splenomegaly, systemic lupus erythematosus, genetically determined thrombocytopenia.
Treatment
Principles of treatment of thrombocytopenia:
1) prevention of bruises and injuries;
2) antibiotics for bacterial infections;
3) transfusion of plasma and large doses of?-globulin;
4) corticosteroids;
5) splenectomy;
6) immunosuppressants (azothiopril, vancristine). Principles of treatment of thrombocytopathies:
1) E-aminocaproic acid, synthetic contraceptives, (bisekurin, microfollin), magnesium sulfate 25% i / m, magnesium thiosulfate inside;
2) topically, s / c or / m adrenochrome monosemicarbazone (adroxon, chromadron, adrenoxyl), dicionon;
3) intravenous administration of platelet mass.
5. Coagulopathy
Coagulopathy is a violation of hemostasis, which is based on a deficiency of certain plasma coagulation factors.
Etiology
Hereditary coagulopathy (correctly called hemophilia) is caused by a genetically determined decrease or change in the plasma components of hemostasis. Acquired coagulopathy occurs with infectious diseases, diseases of the liver and kidneys, severe enteropathies, rheumatoid arthritis and etc.
Classification
Classification of hereditary coagulopathy.
1. Hemophilia: A-deficiency Factor VIII(syntihemophilic globulin); B-deficiency of factor IX (Christmas); C-deficiency of factor XI (the precursor of plasma thromboplastin); D-deficiency XII (Hagemani).
2. Parahemophilia: deficiency of factor V (proaccelerin); factor VII deficiency (proconvertin); factor II deficiency (pro-trolobin); factor X deficiency (Stuart-Prawer).
3. Violation of fibrin formation, deficiency of factor I (fibrinogen). Forms of flow: light, heavy, hidden.
Diagnostics
Clinical diagnostic criteria - pallor of the skin and immune membranes; hemorrhagic syndrome: hamarthrosis, hemorrhage into soft tissues in case of trauma to the skin and mucous membranes (extensive hematomas); hematuria; internal hemorrhages.
Laboratory diagnostic criteria - hematological: anemic syndrome (decrease in the number of erythrocytes and hemoglobin, hypochromia, reticulocytosis with bleeding), hypocoagulation syndrome of blood clotting disorders (according to Lee-White more than 10 minutes), an increase in recalcification time (more than 250 s), an increase in plasma tolerance to heparin (more than 180 s), a decrease in plasma factors.
Survey plan.
1. General analysis of blood, urine.
2. Determination of blood clotting time and platelet count.
3. Coagulogram, determination of antihemophilic globulin (AGG).
4. Radiography of the affected joints.
Differential Diagnosis
Carried out with thrombocytopathies, hemorrhagic vasculitis, with hemarthrosis with rheumatism.
Treatment
The principles of treatment are as follows:
1) an increase in the blood level of AGG: the introduction of AGG concentrates, fresh frozen plasma, concentrates containing factor IX; desmopressin (increasing the level of factor VIII);
2) transfusions, venipuncture against the background of the introduction of epsilon-aminocaproic acid;
3) prevention of injuries and the use of drugs containing aspirin.
And an example of a blood disease caused by a change in the structure and functions of cellular elements is sickle cell anemia, lazy leukocyte syndrome, etc.
Analysis of urine. Conducted to identify concomitant pathology(diseases). Bloodletting is performed in order to normalize the number of blood cells and reduce its viscosity. Before bloodletting is prescribed medications that improve blood flow and reduce its coagulability.
An excess number of red blood cells appears in the blood, but the number of platelets and neutrophilic leukocytes also increases (to a lesser extent). The clinical manifestations of the disease are dominated by manifestations of plethora (plethora) and complications associated with vascular thrombosis. The tongue and lips are bluish-red in color, the eyes are as if bloodshot (the conjunctiva of the eyes is hyperemic). This is due to excessive blood supply and the participation of the hepatolienal system in the myeloproliferative process.
Patients have a tendency to form blood clots due to high viscosity blood, thrombocytosis and changes in the vascular wall. Along with increased clotting blood and thrombus formation in polycythemia, bleeding from the gums and dilated veins of the esophagus is observed. The treatment is based on a decrease in blood viscosity and the fight against complications - thrombosis and bleeding.
Bloodletting reduces blood volume and normalizes hematocrit. The outcome of the disease may be the development of myelofibrosis and cirrhosis of the liver, and with progressive anemia of the hypoplastic type, the transformation of the disease into chronic myeloid leukemia.
Typical examples of blood diseases caused by a change in the number of cellular elements are, for example, anemia or erythremia (increased number of red blood cells in the blood).
Blood disease syndromes
Diseases of the blood system and diseases of the blood are different variants names of the same set of pathologies. But blood diseases are a pathology of its immediate components, such as red blood cells, white blood cells, platelets or plasma.
Hemorrhagic diathesis or pathology of the hemostasis system (blood clotting disorders); 3. Other blood diseases (diseases that do not belong to either hemorrhagic diathesis, or anemia, or hemoblastoses). This classification is very general, dividing all blood diseases into groups based on which general pathological process is the leading one and which cells have been affected by the changes.
The second most common anemia associated with a violation of the synthesis of hemoglobin and erythrocytes is a form that develops with severe chronic diseases. Hemolytic anemia, caused by increased breakdown of red blood cells, are divided into hereditary and acquired.
Anemia syndrome
II A - polycythemic (that is, with an increase in the number of all blood cells) stage. In the general blood test, an increase in the number of erythrocytes, platelets ( platelets), leukocytes (except lymphocytes). Palpation (palpation) and percussion (tapping) revealed an increase in the liver and spleen.
The number of leukocytes (white blood cells, the norm is 4-9x109g / l) can be increased, normal or reduced. The number of platelets (platelets, the adhesion of which ensures blood clotting) initially remains normal, then increases and then decreases again (normal 150-400x109g / l). The erythrocyte sedimentation rate (ESR, a non-specific laboratory indicator that reflects the ratio of blood protein varieties) usually decreases. Ultrasound examination (ultrasound) of the internal organs evaluates the size of the liver and spleen, their structure for damage by tumor cells and the presence of hemorrhages.
Symptoms of diseases of the blood system are quite diverse and most of them are not specific (that is, they can be observed in diseases of other organs and systems). It is precisely because of the non-specificity of symptoms that many patients do not apply for medical care in the first stages of the disease, and come only when there is little chance of recovery. However, patients should be more attentive to themselves and if there are doubts about their own health, it is better not to “pull” and not wait until it “passes by itself”, but immediately consult a doctor.
So let's take a look clinical manifestations major diseases of the blood system.
anemia
Anemia can be an independent pathology or occur as a syndrome of some other diseases.Anemias are a group of syndromes common feature which is a decrease in the level of hemoglobin in the blood. Sometimes anemia is independent disease(hypo- or aplastic anemia, and so on), but more often it occurs as a syndrome in other diseases of the blood system or other body systems.
There are several types of anemia, common clinical sign which is an anemic syndrome associated with oxygen starvation tissues: hypoxia.
The main manifestations of anemic syndrome are as follows:
- pallor of the skin and visible mucous membranes ( oral cavity), nail bed;
- increased fatigue, feeling of general weakness and weakness;
- dizziness, flies before the eyes, headaches, tinnitus;
- sleep disturbances, deterioration or complete lack of appetite, sexual desire;
- shortness of breath, feeling short of breath: shortness of breath;
- palpitations, acceleration of the number of heartbeats: tachycardia.
Manifestations iron deficiency anemia are caused not only by hypoxia of organs and tissues, but also by iron deficiency in the body, the signs of which are called sideropenic syndrome:
- dry skin;
- cracks, ulcerations in the corners of the mouth - angular stomatitis;
- layering, brittleness, transverse striation of nails; they are flat, sometimes even concave;
- burning sensation of the tongue;
- perversion of taste, desire to eat toothpaste, chalk, ashes;
- addiction to some atypical smells: gasoline, acetone and others;
- difficulty swallowing hard and dry food;
- in females - urinary incontinence with laughter, coughing; in children -;
- muscle weakness;
- in severe cases - a feeling of heaviness, pain in the stomach.
B12 and folate deficiency anemia are characterized by the following manifestations:
- hypoxic, or anemic syndrome (signs are described above);
- signs of damage to the gastrointestinal tract (aversion to meat food, loss of appetite, pain and tingling in the tip of the tongue, taste disturbance, "varnished" tongue, nausea, vomiting, heartburn, belching, stool disorders - diarrhea);
- signs of damage to the spinal cord, or funicular myelosis (headache, numbness in the limbs, tingling and crawling, unsteady gait);
- psycho-neurological disorders (irritability, inability to perform simple mathematical functions).
Hypo- and aplastic anemia usually begin gradually, but sometimes debut acutely and progress rapidly. The manifestations of these diseases can be grouped into three syndromes:
- anemic (it was mentioned above);
- hemorrhagic (of various sizes - dotted or in the form of spots - hemorrhages on the skin, gastrointestinal bleeding);
- immunodeficiency, or infectious-toxic (persistent fever, infectious diseases of any organs - otitis media, and so on).
Hemolytic anemia externally manifested by signs of hemolysis (destruction of red blood cells):
- yellow coloration of the skin and sclera;
- an increase in the size of the spleen (the patient notices a formation in the left side);
- increase in body temperature;
- red, black or brown urine;
- anemic syndrome;
- sideropenic syndrome.
Leukemia
With leukemia cancer cells they replace healthy cells in the bone marrow, the deficiency of which in the blood causes the corresponding clinical symptoms. This is a group of malignant tumors that develop from hematopoietic cells. Altered cells multiply in the bone marrow and lymphoid tissue, oppressing and replacing healthy cells, and then go into the blood and are carried with the bloodstream throughout the body. Despite the fact that the classification of leukemia includes about 30 diseases, their clinical manifestations can be grouped into 3 leading clinical and laboratory syndromes:
- syndrome tumor growth;
- tumor intoxication syndrome;
- syndrome of oppression of hematopoiesis.
Tumor growth syndrome occurs due to the spread of malignant cells to other organs and systems of the body and the growth of tumors in them. Its manifestations are as follows:
- swollen lymph nodes;
- enlargement of the liver and spleen;
- pain in bones and joints;
- neurological symptoms (persistent severe headache, nausea, vomiting that does not bring relief, fainting, convulsions, strabismus, unsteady gait, paresis, paralysis, and so on);
- changes in the skin - the formation of leukemids (tubercles white color consisting of tumor cells);
- inflammation of the gums.
The syndrome of tumor intoxication is associated with the release of biologically active substances poisonous to the body from malignant cells, the circulation of cell decay products throughout the body, and changes in metabolism. Its signs are as follows:
- malaise, general weakness, fatigue, irritability;
- decreased appetite, poor sleep;
- sweating;
- increase in body temperature;
- itching of the skin;
- weight loss;
- pain in the joints;
- renal edema.
The syndrome of oppression of hematopoiesis occurs due to a lack of red blood cells (anemic syndrome), platelets (hemorrhagic syndrome) or leukocytes (immunodeficiency syndrome) in the bloodstream.
Lymphomas
Malignant is a group of tumors of the lymphatic system arising from the formation of a pathologically altered lymphoid cell capable of uncontrolled proliferation (reproduction). Lymphomas are usually divided into 2 large groups:
- Hodgkin's (Hodgkin's disease, or lymphogranulomatosis);
- non-Hodgkin's lymphomas.
Lymphogranulomatosis- This is a tumor of the lymphatic system with a primary lesion of the lymphoid tissue; is about 1% of all oncological diseases adults; more often people aged from 20 to 30 and over 50 suffer.
Clinical manifestations of Hodgkin's disease are:
- asymmetric enlargement of the cervical, supraclavicular or axillary lymph nodes (the first manifestation of the disease in 65% of cases); the nodes are painless, not soldered to each other and to the surrounding tissues, mobile; with the progression of the disease, the lymph nodes form conglomerates;
- in every 5th patient, lymphogranulomatosis debuts with an increase in the lymph nodes of the mediastinum, which is initially asymptomatic, then cough and pain behind the sternum appear, shortness of breath);
- a few months after the onset of the disease, symptoms of intoxication appear and steadily progress (fatigue, weakness, sweating, loss of appetite and sleep, weight loss, skin itching, fever);
- propensity to infections of viral and fungal etiology;
- all organs containing lymphoid tissue- there are pains in the sternum and other bones, the liver and spleen increase in size;
- in the later stages of the disease, signs of anemic, hemorrhagic syndromes and a syndrome of infectious complications appear.
Non-Hodgkin's lymphomas- This is a group of lymphoproliferative diseases with primary localization mainly in the lymph nodes.
Clinical manifestations:
- usually the first manifestation is an increase in one or more lymph nodes; when probing, these lymph nodes are not soldered to each other, painless;
- sometimes, in parallel with an increase in lymph nodes, symptoms of general intoxication of the body appear (weight loss, weakness, itching of the skin, fever);
- a third of patients have lesions outside the lymph nodes: in the skin, oropharynx (tonsils, salivary glands), bones, gastrointestinal tract, lungs;
- if the lymphoma is localized in the gastrointestinal tract, the patient is worried about nausea, vomiting, heartburn, belching, pain in the abdomen, constipation, diarrhea, intestinal bleeding;
- sometimes lymphoma affects the central system, which is manifested by severe headaches, repeated vomiting that does not bring relief, convulsions, paresis and paralysis.
multiple myeloma
One of the first manifestations of myeloma is persistent bone pain. multiple myeloma, or multiple myeloma, or plasmacytoma is a separate type of tumor of the blood system; comes from precursors of B-lymphocytes that retain a certain ability to differentiate.
Main syndromes and clinical manifestations:
- pain syndrome (pain in the bones (ossalgia), radicular pain between the ribs and in the lower back (neuralgia), pain in the peripheral nerves (neuropathy));
- syndrome of destruction (destruction) of bones (pain in the area of bones associated with osteoporosis, compression fractures of bones);
- hypercalcemia syndrome ( high content calcium in the blood - manifested by nausea and thirst);
- hyperviscose, hypercoagulable syndrome(due to a violation of the biochemical composition of the blood - headaches, bleeding, thrombosis, Raynaud's syndrome);
- recurrent infections (due to immunodeficiency - recurring tonsillitis, otitis media, pneumonia, pyelonephritis, and so on);
- renal failure syndrome (edema that occurs first on the face and gradually spreads to the trunk and limbs, increased blood pressure, not amenable to correction by conventional antihypertensive drugs, turbidity of urine associated with the appearance of protein in it);
- in the later stages of the disease - anemic and hemorrhagic syndromes.
Hemorrhagic diathesis
Hemorrhagic diathesis is a group of diseases, a common feature of which is increased bleeding. These diseases may be associated with disorders in the blood coagulation system, a decrease in the number and / or function of platelets, pathology of the vascular wall, and concomitant disorders.
Thrombocytopenia- decrease in platelet count peripheral blood less than 140*10 9 / l. Main feature this disease- hemorrhagic syndrome of varying severity, directly dependent on the level of platelets. Usually the disease is chronic, but it can also be acute. The patient pays attention to spot rashes that appear spontaneously or after injuries, subcutaneous hemorrhages on the skin. Blood seeps through wounds, injection sites, surgical sutures. Nosebleeds, bleeding from the digestive tract, hemoptysis, hematuria (blood in the urine) are less common, in women - heavy and prolonged menstruation. Sometimes the spleen is enlarged.
Hemophilia- This hereditary disease, characterized by a violation of blood clotting due to a lack of one or another internal clotting factor. Clinically
