Immune hemolytic anemia. Hereditary hemolytic anemia associated with impaired activity of erythrocyte enzymes
Immune hemolysis occurs due to the formation of antibodies to erythrocyte antigens, resulting in the destruction of erythrocytes by phagocytosis or complement activation. Immune hemolysis can be caused by both autoantibodies and alloantibodies. Immune hemolysis is also distinguished as intravascular And extravascular(macrophages act as effectors of extravascular immune hemolysis).
Isoimmune hemolytic anemias arise as a result of the ingestion from the environment of antibodies to the antigens of the patient's erythrocytes or erythrocytes containing antigens against which the patient has antibodies (for example, hemolytic disease of the newborn).
Alloimmune hemolytic anemia
Alloimmune hemolytic anemias result from hemolysis during transfusion of red blood cells that are incompatible in terms of blood type (AB0 system), Rh factor, or some other system against which antibodies have been developed in the patient's body. Transfusion reactions are mild and severe.
Severe transfusion reactions occur when antibodies of the IgM class interact with erythrocyte antigens A and B, resulting in complement activation and intravascular hemolysis. Intravascular hemolysis is accompanied by the release of free hemoglobin into the blood plasma, hemoglobinuria and the formation of methhemalbumin (brown pigment). When transfused with an incompatible red blood cell mass, the recipient's body temperature rises, chills, pain in the chest and back (see article). Such symptoms can occur even with transfusion of a small amount of red blood cells. Severe, life-threatening complications may occur: acute renal failure, DIC, and shock. If a transfusion reaction occurs, the prognosis depends on the volume of the transfused erythrocyte mass and the titer of antibodies to erythrocyte antigens A and B in the blood of the recipient.
If during a blood transfusion the recipient shows signs of a transfusion reaction, the transfusion procedure should be stopped immediately. Then, samples of the recipient's blood and donor red blood cells should be taken for analysis (culture and microscopic examination). The container with the used donated red blood cell mass is not thrown away - it is sent to the blood transfusion center together with the recipient's blood sample for testing. individual compatibility, direct Coombs test and re-determination of blood group and Rh factor.
Autoimmune hemolytic anemia
Autoimmune hemolytic anemias are characterized by the release of antibodies to their own unchanged erythrocyte antigens.
Autoimmune hemolytic anemia with incomplete heat agglutinins (antibodies). Almost all warm antibodies belong to the IgG class, some to IgA, rarely to the M class. Hemolysis of erythrocytes by IgG antibodies can occur by two mechanisms:
- Immune adhesion of erythrocytes to macrophages mediated directly by antibodies and complement components adsorbed on the erythrocyte membrane is the main mechanism
- Complement activation, which completes the damage to the erythrocyte membrane.
Autoimmune hemolytic anemia with warm antibodies can occur at any age (more common among adult women). In about 25% of patients, this pathology occurs as a symptom of hemoblastoses or systemic diseases connective tissue(especially in SLE - systemic lupus erythematosus). severe forms autoimmune hemolytic anemia are characterized by acute onset: fever, weakness, jaundice. chronic course due to severe hemolysis, it is accompanied by an increase in the spleen and in almost 50% of patients - an increase in the liver. It may also occur immune thrombocytopenia - Fisher-Evans syndrome(the patient develops antibodies to erythrocytes and platelets, sometimes vein thrombosis occurs).
Laboratory studies demonstrate the presence of normochromic anemia, reticulocytosis (Figure 1, 2), the level of leukocytes is determined by the pathology against which autoimmune hemolytic anemia developed, the platelet level remains normal or thrombocytopenia is observed.
Figure 1. Hemolytic anemia. Bone marrow punctate. Strengthening of the erythroid process (circling according to Romanovsky-Giemsa, magnification ×50)
Figure 2. Hemolytic anemia. Bone marrow punctate. Erythrophage. Strengthening of the erythroid process (circling according to Romanovsky-Giemsa, magnification ×50)
The myelogram shows irritation of the "red germ" (Figure 3-7).
Figure 3. Hemolytic anemia. Bone marrow punctate. Strengthening of the erythroid process. Basophilic normoblasts (Romanovsky-Giemsa stain, magnification ×100)
Figure 4. Hemolytic anemia. Reticulocytosis in peripheral blood(surrounded with brilliant cresyl blue, magnification ×100)
Figure 5. Hemolytic anemia. Bone marrow(environment according to Romanovsky-Giemsa, enlargement ×50)
Figure 6. Hemolytic anemia. Bone marrow. Erythroid islet
Figure 7. Hemolytic anemia. Reticulocytes of varying degrees of maturity in peripheral blood (stained with brilliant cresyl blue, uv ×100)
There is also an increase in the acid resistance of erythrocytes and a decrease in their osmotic resistance. Level increases indirect bilirubin. In about 98% of cases, a direct Coombs test shows a positive result. Most often, immunoglobulin G is found in combination with or without the C3 complement component. If a direct Coombs test showed negative result, analysis for the presence of antibodies to erythrocytes is carried out by aggregate-hemagglutation test. It is important to note that even in severe immune hemolytic anemia (as in mild form), antibodies may not be detected.
Autoimmune hemolytic anemia with thermal hemolysins is a rare pathology characterized by a calm onset (with rare exceptions). The disease is accompanied by intravascular hemolysis (the patient's urine becomes black or brown), thrombosis of mesenteric vessels and peripheral veins is possible. Some patients have an enlarged spleen and/or liver.
Laboratory tests show high level unbound hemoglobin in the blood, a negative Coombs test and a positive Hem test, which determines the degree of sensitivity to the action of complement.
Autoimmune hemolytic anemia with complete cold agglutinins. Cold agglutinins include mainly immunoglobulin M, sometimes a mixture of immunoglobulins of different classes that can cause maximum agglutination of erythrocytes at a temperature of +4°C. In a low titer (no more than 1:64), cold agglutinins are also found in healthy individuals (as a rule, these are polyclonal cold agglutinins). The symptomatic form of this disease is observed in infectious mononucleosis, influenza, adenovirus infection, mycoplasma pneumonia, chronic hepatitis, hemoblastoses. Cold antibodies, which react mainly on red blood cells in adults (anti-I antibodies), are characteristic of mycoplasmal infections and benign monoclonal gammopathy. Anti-I antibodies are antibodies that react predominantly with fetal red blood cells. They are typical for infectious mononucleosis and high-grade lymphomas.
It is necessary to distinguish cold from cryoglobulins. Cold agglutinins- immunoglobulins that bind to erythrocyte antigens at temperatures below 37°C. Cryoglobulins, unlike cold agglutinins, precipitate when low temperatures and do not bind to erythrocyte antigens.
Laboratory studies show anemia and reticulocytosis. Agglutination of erythrocytes at room temperature is strongly pronounced (it is even impossible to count the number of erythrocytes). In the blood, the level of indirect bilirubin rises, cold agglutinins are detected. A direct Coombs test with complement antibodies may be positive, with antibodies to immunoglobulins, as a rule, it is negative.
In the treatment of thermal forms of autoimmune hemolytic anemia, the first-line drug is Prednisolone. In case of absence therapeutic effect and the occurrence of various complications from glucocorticosteroid therapy, the patient undergoes surgical removal of the spleen (splenectomy). In some cases, the patient is prescribed Cyclosporin A.
Immune hemolytic anemias are a heterogeneous group of anemias characterized by the involvement of immunoglobulins (G and M) or immune lymphocytes in the damage and premature death of erythrocytes or erythrokaryocytes.
Among immune hemolytic anemias, there are following groups:
1) autoimmune;
2) alloimmune;
3) heteroimmune.
Autoimmune hemolytic anemia characterized by the presence of antibodies against its own unchanged erythrocytes. This group of anemia includes hemolytic anemia caused by warm antibodies, cold antibodies; hemolytic anemia caused by biphasic hemolysins and incomplete thermal agglutinins.
Heteroimmune hemolytic anemias (drugs). Autoantibodies (IgG, IgM) may appear when taking certain drugs - antibiotics, streptomycin, indomethacin, anti-tuberculosis drugs, phenacytin, quinidine, etc. The mechanism for the development of drug-induced immunohemolytic anemia may be different. The drug can interact with the components of the erythrocyte membrane and stimulate the formation of medicinal antibodies of the IgG type. This is the mechanism of action of penicillin. The drug can participate in the formation of an immune complex with an antibody (IgM), settle on the erythrocyte membrane, activate complement and cause hemolysis of the cell. In some cases, the drug substance can induce the formation of autoantibodies, such as in warm autoimmune hemolytic anemia (IgG, IgM). A similar mechanism was found in a‑methyldopa, mebedrol, and elenium.
Isoimmune (alloimmune) hemolytic anemias. They develop in newborns with incompatibility of the AB0 systems and Rh of the fetus and mother (hemolytic disease of the fetus and newborn), as well as a complication of blood transfusions that are incompatible with the AB0 systems, Rh and its rare varieties.
Hemolytic disease of the newborn. The disease occurs due to the antigenic difference between the erythrocytes of the mother and the child, the production of antibodies by the mother's immunocompetent system, the penetration of anti-erythrocyte antibodies (IgG) through the placenta and the destruction of the erythrocytes of the fetus and newborn.
There are three clinical forms diseases: anemic, icteric and edematous. Pathology is detected from the moment the child is born or in the first hours of life.
Clinical picture hemolytic disease largely determined by the amount of antibodies that cross the placenta, however great importance has a degree of maturity of the body of the newborn (a more severe course of the disease was noted in premature babies).
A high antibody titer causes intense hemolysis of erythrocytes, which begins even in the period prenatal development and intensifies at the time of the birth of the child.
Blood picture. The number of erythrocytes decreases to 2-3 million or less, the hemoglobin content in severe cases is 60-80 g/l. Significantly increased the number of reticulocytes - 10-15%. Erythroblasts appear in the peripheral blood, normocytes of the CP are either within the normal range or slightly higher. On the part of white blood, neutrophilic leukocytosis with a shift to the left.
One of the most dangerous symptoms of hemolytic disease of the newborn is kernicterus with symptoms of injury nervous system- bilirubin encephalopathy. Violation of bilirubin metabolism is aggravated by the imperfection of the processes of conjugation of indirect bilirubin due to the immaturity of the enzyme uridine diphosphoglucuronyl transferase in the liver. Along with a violation of bilirubin metabolism, violations of the protein and prothrombin-forming functions of the liver are detected early, which leads to hemorrhages and bleeding.
The content of the article
Hemolytic anemia - pathological process caused by accelerated hemolysis of erythrocytes.Etiology and pathogenesis of hemolytic anemia
The causes of increased hemolysis of erythrocytes in the vast majority of cases are hereditary defects in the enzyme systems of erythrocytes, mainly glycolysis enzymes, membrane structure, and violations of the amino acid composition of hemoglobin. All these reasons cause less resistance of erythrocytes and their increased destruction. immediate cause hemolysis can be infectious, medicinal and toxic effects, realizing increased hemolysis of erythrocytes with their functional, and sometimes morphological inferiority. In some cases (with diffuse diseases of the connective tissue, acute immune processes that have arisen during infectious disease or after prophylactic vaccination) an autoimmune process occurs with the formation of antibodies to erythrocytes that agglutinate erythrocytes.Classification of hemolytic anemia
The classification of hemolytic anemia has not been definitively developed. The following can be used as a working classification.1. Hereditary hemolytic anemia associated with a defect in the erythrocyte membrane.
2. Hereditary hemolytic anemia associated with impaired activity of erythrocyte enzymes.
3. Hereditary hemolytic anemia associated with a violation of the structure or synthesis of hemoglobin.
4. Acquired hemolytic anemia (immune, infectious, toxic).
Hemolytic anemia is characterized by the following clinical and laboratory signs. In connection with the increased destruction of erythrocytes develops varying degrees severity of anemia and jaundice.
As a rule, jaundice develops against the background of severe pallor of the skin (pale jaundice). With significant hemolysis, feces, sometimes urine, can be intensely stained. In connection with the increased excretion of bilirubin conversion products, the liver may increase, an increase in the spleen, which is the site of the breakdown of red blood cells, is noted. Hematologically, anemia of the normochromic type is detected with a pronounced regenerative reaction (reticulocytosis, sometimes significant - up to 8-10% or more), in some cases, single normoblasts appear in the peripheral blood. The change in the size, shape and osmotic resistance of erythrocytes depends on the form of the disease. In the blood, there is an increase in the level of indirect bilirubin, in the urine - increased amount urobilin, in feces - stercobilin. In the study of bone marrow punctate - a pronounced erythronormoblast reaction.
Hereditary hemolytic anemia associated with a defect in the erythrocyte membrane
Hereditary-familial microspherocytic anemia of Minkowski - Chauffard, as a rule, is observed in several family members. The type of inheritance is autosomal dominant. The probability of disease in offspring is 50%. The disease is based on the loss of lipids by erythrocytes, as a result of which the membrane surface is reduced. Erythrocytes take the form of a microspherocyte (the diameter of erythrocytes decreases to 5 - 6 microns, normally 7 - 7.5 microns, their life expectancy is significantly reduced and rapid hemolysis occurs.The disease proceeds in the form of severe hemolytic crises, sometimes hemolysis can be constant or undulating, somewhat accelerated. The appearance of patients is sometimes typical of hereditary diseases- square skull, deformed auricles, "Gothic" palate, strabismus, dentition disorder, extra fingers, etc. With this form of anemia, there is a significant increase in the spleen. When examining blood, there is a decrease in the number of erythrocytes, reticulocytosis, a decrease in the osmotic resistance of erythrocytes.
The amount of indirect bilirubin is increased and is 26-43 µmol/l in mild cases and 85-171 µmol/l in severe forms.
hereditary ovalocytosis- hemolytic anemia of moderate severity, occurring without hemolytic crises (in children of the first months of life, hemolytic crises may occur), with moderate pallor and icterus of the skin and mucous membranes. In some cases, the family nature of the disease is established. On hematological examination - 80 - 90% of ovalocytes (erythrocytes oval shape), moderate anemia (3.5 - 3.8 T / l of erythrocytes) with good regenerative capacity of the bone marrow (reticulocytes up to 5% or more).
Hereditary stomatocytosis- a rare form of morphological immaturity of erythrocytes. Clinically, the disease proceeds in the form of moderately severe anemia, followed by jaundice and splenomegaly. Osmotic resistance of erythrocytes is increased.
Pediatric pycnocytosis, apparently not hereditary, but a transient inferiority of erythrocytes in children during the first months of life, causing their increased destruction. Pycnocytes - erythrocytes with jagged edges(numerous sharp branches). Clinically, the disease manifests itself when the number of pycnocytes is 40-50% or more. The disease usually occurs in the first weeks of life.
Hereditary hemolytic anemia associated with impaired activity of erythrocyte enzymes
The process is based on a violation of various enzyme systems of the erythrocyte - glucose-6-phosphate dehydrogenase (G-6-PD), pyruvate kinase, glutathione-dependent enzymes. The disease often has a familial character with a dominant way of transmitting the trait. Sometimes the family character is not established. Hemolysis passes by the type of chronic, without severe hemolytic crises. With a deficiency of G-6-PD, hemolysis may first occur in children under the influence of intercurrent diseases and after taking medications (sulfonamides, salicylates, nitrofurans). There is pallor, icterus of the skin and mucous membranes, "anemic" noise over the region of the heart, moderate hepatosplenomegaly. In the study of blood - a decrease in the number of erythrocytes, high reticulocytosis, an increase in the level of indirect bilirubin.There is no microspherocytosis, erythrocytes are of normal size and shape or are somewhat altered (such as macrocytes of a round or somewhat oval shape). Osmotic resistance of erythrocytes is normal.
Hereditary hemolytic anemia associated with a violation of the structure or synthesis of hemoglobin
The protein part of hemoglobin - globin has a complex structure and includes 574 amino acids. Currently, about 50 variants of hemoglobin are known, depending on its physical and chemical properties and amino acid composition. IN normal conditions From 6-8 months of age, hemoglobin consists of three fractions: HbA (adultus - adult) makes up the main part, HBF (foetus - fetus) - 0.1 - 0.2%, HbA - 2 - 2.5%. At birth, the majority is HBF - 70 - 90%. Other types of hemoglobin are pathological.Influenced by a number of factors external environment fixed hereditarily, may change amino acid composition hemoglobin. In this case, pathological varieties of hemoglobin arise - hemoglobin C, D, E, G, H, K, L, M, O, S, etc. At present, the symptom complexes associated with the presence of normal, but characteristic of the fetus HBF, as well as diseases associated with HBS, HBC, HBE, HBD and a combination of various pathological forms of hemoglobin, have been studied in detail. It should be noted that hemoglobinopathy is widespread in a number of areas of the globe, especially in Africa, on the coast mediterranean sea as well as in tropical regions South-East Asia and among certain populations in North and Central America.
Thalassemia(congenital leptocytosis, target cell anemia, Mediterranean anemia, Cooley's anemia). The disease was first described by Cooley and Lee in 1925 in the population of the coastal regions of the Mediterranean Sea, from which it got its name (from the Greek thalassa - sea). The process is based on increased synthesis of fetal hemoglobin in amounts not characteristic of the body child older than a year and adults (up to 80 - 90%). Thalassemia is hereditary disorder formation of hemoglobin.
Clinically, the disease is characterized by severe progressive hemolysis in thalassemia major or milder hemolysis in thalassemia minor, with the development of anemia, hepatosplenomegaly. A pronounced picture of the disease develops at the age of 2-8 years. Anomalies of development are often observed. On hematological examination, typical target-cell erythrocytes.
sickle cell anemia(drepanocytosis) refers to diseases in which pathological HBS is synthesized instead of normal HvA, which differs from HvA in that the glutamic acid molecule in globin is replaced by a valine molecule. As a result, it changes electric charge hemoglobin, which determines its colloidal state, the possibility of changing forms, gluing and hemolysis of erythrocytes. These properties are most pronounced under hypoxic conditions. specific feature of this disease is the formation of crescent-shaped erythrocytes with a decrease in tension (partial pressure) of oxygen in environment which leads to hemolysis.
The course of the disease is with frequent hemolytic crises. Characteristic symptoms: jaundice, splenomegaly, delayed physical development.
Acquired hemolytic anemia of immune origin
Sometimes it can be observed in diffuse connective tissue diseases, most often in systemic lupus erythematosus ( autoimmune form). In the neonatal period, isoimmune hemolytic anemia occurs due to Rh-conflict or incompatibility of the blood of the mother and fetus in the main groups of the ABO system.Diagnosis established on the basis of clinical data, laboratory research and family history studies.
Treatment. At hemolytic crisis prescribe intravenous fluid (5% glucose solution, Rpnger solution), blood plasma, vitamins, according to indications steroid hormones, antibiotics. Showing drugs that favorably affect carbohydrate (cocarboxylase, ATP, thiamine) and protein (anabolic hormones, etc.) metabolism.
With microspherocytosis, splenectomy is a highly effective measure. Indications: the presence of constant or in the form of crises of anemia, significant hyperbilirubinemia, developmental delay.
Blood transfusions are carried out only for health reasons during severe crises, with deep anemia. Steroid therapy is recommended in the development of aplastic crises. The prognosis is favorable. Hereditary forms associated with erythrocyte abnormality, special treatment do not require.
In thalassemia, it is advisable to prescribe folic acid, which is necessary for the bone marrow in in large numbers due to ineffective erythropoiesis. The use of blood transfusions gives a temporary effect. The use of Desferal is recommended.
At sickle cell anemia during a crisis, the patient must be placed in warm room, since at low temperatures the degree of sickle cellity increases. The use of agents aimed at the prevention of thrombosis (magnesium sulfate, acetylsalicylic acid) is recommended.
In children older than 1 year, acquired hemolytic anemia can be immunopathological, autoimmune or heteroimmune.
In older children and adults, it often occurs as a result of a breakdown in immunological tolerance to an antigen as a result of various chronic diseases (lymphocytic leukemia, nonspecific ulcerative colitis and other diseases).
It is often symptomatic.
Antibodies can damage peripheral blood or bone marrow erythrocytes.
In connection with different types of antibodies, autoimmune hemolytic anemias are divided into:
Autoimmune hemolytic anemia (AIHA) with incomplete cold agglutinins;
AIHA with complete cold agglutinins;
AIHA with thermal hemolysins;
AIGA with biphasic hemolysins.
Cold agglutinins (complete and incomplete) cause agglutination of red blood cells in the body (test tube) when the temperature drops.
In these cases, erythrocytes stick together, damage their membranes. Incomplete antibodies are fixed on red blood cells, causing them to stick together.
The bone marrow in autoimmune hemolytic anemia is in the stage of irritation of the bone sprout with high megacoryocytosis, reticulocytosis is common.
At the same time, during the study of blood serum, an increase in the content of gamma globulins is determined, except for hyperbilirubinemia, morphologically, erythrocytes do not change, the level of reticulocytes is high, erythrokaryocytes can be determined. In blood smears, erythrocytes with "corroded" edges are noted.
Main clinical symptoms
Reduced to the appearance of signs of anemic syndrome. In the case of a phonic course of the disease, along with anemia, there is a slightly pronounced jaundice. When hemolysis occurs in other cases, anemia and jaundice rapidly increase, which is often accompanied by fever.
There is an enlargement of the spleen.
In the variant with the phenomena of intravascular coagulation and autoimmune hemolytic anemia, erythrocytes damaged by autoantibodies are absorbed by macrophage cells. With this form, there is a discharge of dark urine.
Autoimmune anemia can occur in old age. If this is associated with the occurrence of cold agglutinins, the disease develops against the background of " full health»: suddenly there is shortness of breath, pain in the region of the heart, lower back, the temperature rises, jaundice appears. In other cases, the disease is manifested by pain in the abdomen, joints, subfebrile temperature.
The chronic course often takes the form of intracellular hemolysis caused by cold agglutinins, which is a consequence of sudden cooling.
In such cases, patients do not tolerate cold, gangrene of the fingers may develop.
Patients often have intolerance to cold, when exposed to it, blue fingers, ears, and the tip of the nose occur, pain in the limbs occurs, and the spleen and liver increase.
Diagnostics
It is built on the basis of signs of hemolysis, staging serological reactions, direct and indirect sample Coombs, incubation of erythrocytes and serum under various temperature conditions.
Glucocorticoids are prescribed. With the ineffectiveness of steroid therapy, the issue of splenectomy is decided. In cases of autoimmune hemolytic anemia with complete cold agglutinins, along with corticosteroids, immunosuppressants (cyclophosphamide, methotrexate, etc.) are prescribed. At severe course blood or “washed” (frozen) red blood cells are transfused.
the causative agents of malaria, barto-
nelleze and clostridiosis. In some patients, hemolysis was also caused by other microorganisms, including many gram-positive and Gram-negative bacteria and even the causative agents of tuberculosis. Hemolytic disorders can cause viruses and mycoplasmas, but apparently indirectly through immunological mechanisms.
Immune hemolytic anemia
Immune hemolytic anemia caused by warm antibodies
Warm antibodies causing hemolytic anemia may occur primarily (idiopathically) or as a secondary phenomenon in various diseases (Table 24). Such anemia is more common in women, and the frequency of secondary forms increases with age. Autoimmune hemolytic anemia appears to occur in the presence of a genetic predisposition and immunological dysregulation. When searching for the causes of autoimmune hemolytic anemia in the elderly, one should first think about secondary forig or drug etiology.
Table 24. Immune hemolytic anemia
Associated with warm antibodies
a) idiopathic autoimmune hemolytic anemia
b) secondary at:
systemic lupus erythematosus and other collagenoses chronic lymphocytic leukemia and other malignant lymphoreticular diseases, including multiple myeloma other tumors and malignancies
viral infections immunodeficiency syndromes
Associated with cold antibodies
a) primary - idiopathic "cold agglutinin disease"
b) secondary at:
infections, especially mycoplasma pneumonia, chronic lymphocytic leukemia, lymphomas
c) paroxysmal cold hemoglobinuria
idiopathic secondary in syphilis and viral infections
Drug-induced immune hemolytic anemia
a) penicillin type
b) stibophenone type (type of "innocent witness")
c) type of conditioned a-methyldopa
d) streptomycin type
Warm antibody autoimmune hemolytic anemia is caused by different reasons and proceeds differently. Forms of anemia secondary to malignant neoplasms, usually develop gradually, and their course corresponds to the course of the underlying disease. Primary forms of anemia are very variable in their manifestations - from mild, almost asymptomatic to fulminant and fatal. Symptoms are usually those of anemia and include weakness and dizziness. TO
typical features include hepatomegaly, lymphadenopathy, and especially splenomegaly, but jaundice is usually not seen.
.
Diagnosis of autoimmune hemolytic anemia is based mainly on laboratory data. Normocytic normochromic anemia is usually found, but sometimes it is macrocytic, depending on the degree of reticulocytosis. The number of reticulocytes is usually increased, but comorbidities - anemia associated with chronic diseases, scarce state or myelophthisis can significantly reduce the severity of reticulocytosis.
In about 25% of cases, reticulocytopenia is observed, apparently due to antibodies to reticulocytes. On a peripheral blood smear classic cases microspherocytosis, poikilocytosis, polychromatophilia, anisocytosis and polychromatophilic macrocytes are found. Nucleated erythrocytes are often found. The white blood cell count may be low, normal, or increased (with acute development anemia); the platelet count is usually within the normal range. The simultaneous presence of autoimmune hemolytic anemia and autoimmune thrombocytopenia is characteristic of Evans syndrome, which can
may accompany lymphoma.
Serum bilirubin is usually slightly elevated, and hemolysis is usually extravascular.
Coombs test. Positive results of a direct antiglobulin test indicate the presence of antibodies on the surface of red blood cells, which is typical for almost all patients with autoimmune hemolytic anemia.
This test can be modified to provide information on immunoglobulin class and subclass, and the presence of complement components. Indirect detection can be used to detect antibodies in serum. antiglobulin test. Theoretically, the only drawback of the Coombs test is its relatively low sensitivity. Commercial reagents commonly used in blood bank laboratories give positive reactions if there are 100-500 antibody molecules on the surface of each red blood cell. It should be remembered that since 10 molecules of anti-Rh antibodies are sufficient to reduce the half-life of erythrocytes to 3 days, severe hemolytic anemia may occur in patients with negative an-
tiglobulin test, but this situation is rare. Currently used
or polybrene into a suspension of erythrocytes in order to reduce the distance between them. In particular, the use of polybrene in automatic analyzers with flow systems has significantly increased the sensitivity of the method. Much more sensitive and widely used methods with the treatment of erythrocytes proteolytically
mi enzymes.
In autoimmune hemolytic anemia caused by warm antibodies, in 30-40% of patients, only IgG antibodies are found on erythrocytes, in 40-50% - IgG and complement, and in 10% - only complement (usually in patients with systemic lupus erythematosus). Many antibodies are directed against antigenic determinants of Rh, which makes it difficult to determine the blood group and compatibility. Antibodies of the IgG class are usually polyclo-
nal.
Therapy for autoimmune hemolytic anemia due to warm antibodies must necessarily include
treatment of the underlying disease. If the underlying disease is lymphoma and especially - chronic lymphocytic leukemia or tumor, treatment of it in many cases leads to remission of hemolytic anemia. In emergency situations, lightning-fast development hemolysis may require a blood transfusion. At the same time, however, one must remember about the problems associated with determining the group affiliation and compatibility of blood. In these cases, the "most compatible" RBCs are used for transfusion. Transfusion is not enough compatible blood should be carried out slowly, constantly monitoring the patient's condition. At the same time, adreno-corticosteroids should be administered.
These hormones are the drugs of choice at the start of treatment. Usually start with prednisolone at a dose of 40 mg/m2 of body surface per day, but higher doses may be required. Improvement in hematological parameters usually occurs on the 3rd-7th day and in the following weeks the level of hematological
can be gradually reduced. As a rule, the dose should be halved over 4-6 weeks, and then slowly discontinued.
zolon in the next 3-4 months. At-
Corticosteroids have no effect in about 15-20% of patients, which is why it is necessary to resort to splenectomy or the appointment of cytotoxic drugs. In about a quarter of cases, the corticosteroid can be completely canceled, and in the remaining cases, maintenance doses of steroids must be used, despite the risk of associated complications in the elderly.
Splenectomy is indicated in cases where anemia does not respond to steroid treatment, if long-term use of high doses of steroids is required, and if serious complications steroid therapy. The effectiveness of splenectomy increases with the selection for surgery of those patients in whose spleen erythrocytes labeled with 51 Cr are intensively retained. The question of the expediency of splenectomy in this elderly patient should always be decided taking into account all the diseases he has. Before the opera
The patient should be given a pneumococcal vaccine to reduce the risk of postoperative pneumococcal sepsis.
Cytotoxic drugs in the elderly are prescribed only in cases where there is no effect of treatment with corticosteroids or splenectomy, as well as in cases of recurrence of hemolytic anemia after splenectomy or in the presence of contraindications to this operation. The most commonly used drugs are cyclophosphamide and azathioprine (both in combination with prednisone).
Immune hemolytic anemia caused by cold antibodies
Autoantibodies that react with red blood cells at temperatures below 32°C are called cold autoantibodies. They are responsible for the development of two clinical syndromes: syndrome of "cold agglutinins" and paroxysmal cold hemoglobinuria (Table 24). Last state very rare, usually with syphilis.
Cold | agglutinins, like | relate | |||
the IgM class. These | antibodies | ||||
be both polyclonal and monoclonal (Table 25), | |||||
and nearly all bind complement. Pain- | |||||
Most antibodies are specific for one of the erythrocytes. | |||||
cytic antigens II. II antigens are also present on other | |||||
cells, so | cold anti-II agglutinins can | ||||
Table 25 | Diseases leading to cold |
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new agglutinins | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Polyclonal cold agglutinins | Monoclonal cold agglutinins |
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Chronic cold agglutination disease |
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Pneumonia caused by mycoplasmas | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Waldenstrom's macroglobulinemia |
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Angioimmunoblastic lymphadenopathy | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Collagenoses and immunocomplex diseases | Chronic lymphocytic leukemia |
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Subacute bacterial endocarditis | Kaposi's sarcoma |
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Other infections | multiple myeloma |
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Mycoplasma pneumonia (rare) |
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Polyclonal variant of cold agglutination disease |
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new" most often | due to Mycoplasma pneumoniae infection |
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and observed | mostly in young people | patients, but may also occur in the elderly. Other diseases that produce polyclonal cold agglutinins are rare. However, hemolytic anemia due to monoclonal cold agglutinins is observed mainly in the elderly, and its frequency reaches a maximum in age group 60-80 years old Cold agglutinins, associated associated with malignant lymphoreticular neoplasms, also occurs almost exclusively in the elderly Clinical manifestations due to intravascular cell agglutination or hemolysis. When blood passes through the capillaries of the skin and subcutaneous tissues, its temperature can drop to 28 ° C or even lower. If cold antibodies are active at this temperature, they agglutinate cells and bind complement. Agglutination leads to vascular occlusion, and complement activation can cause intravascular hemolysis and cell sequestration in the liver Acrocyanosis or a pronounced discoloration of the skin - from pale to cyanotic - is due to intracapillary agglutination of red blood cells in those parts of the body that are cooled. or pain and are most often observed in the distal sections of co- Chronic hemolytic anemia in idiopathic cold agglutinin disease is usually mild and is characterized by extravascular hemolysis. The hemoglobin concentration is usually maintained above 70 g/l. In many cases, the patient's condition worsens cold weather. The C3 b inactivator system may be functionally deficient under cold stress, high antibody titer, or high thermosetting. The development of acute intravascular hemolysis due to cooling may be accompanied by hemoglobinuria, chills, and even acute renal failure. Ehrlich's finger test can be used to detect hemolysis on cooling. The finger is pulled with a rubber cuff so as to block venous return, and immersed in cold water (20 ° C) for 15 minutes. For control, the other finger is immersed in water having a temperature of 37 ° C. After centrifugation of a blood sample from a finger that was in cold water, revealing hemolysis occurs; blood taken from a finger warm water, is not hemolyzed. The patient usually has acrocyanosis, pallor, and sometimes mild jaundice. The spleen is rarely palpated with difficulty, and the liver may also be slightly enlarged. A blood test reveals signs of anemia, moderate reticulocytosis and sometimes mild hyperbilirubinemia, as well as specific manifestations of intravascular hemolysis. Blood cells can agglutinate at room temperature, and the first suggestion of a possible diagnosis arises from difficulties in counting the number of red blood cells or in preparing a peripheral blood smear. The diagnosis is confirmed by the detection of elevated titers of cold agglutinins. The antiglobulin test is positive, but specific only for complement components, while the test with antigammaglobulin serum is negative. In severe hemolysis, complement levels are reduced. Treatment given state is mainly to give the patient advice on how to keep the body temperature above that at which antibodies show their activity. Blood transfusions are not usually necessary and may even be dangerous due to the possible increase in hemolysis. If it is nevertheless necessary to transfuse blood, then the compatibility test should be carried out at 37 ° C, and donated blood should not be transfused before transfusion.
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2023 "kingad.ru" - ultrasound examination of human organs
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