Hemolytic anemia - is clinical and hematological syndrome that occurs as a result of shortening the duration of the functioning of red blood cells due to their increased decay.This pathology combines a group of diseases inherited and acquired character in the pathogenesis of which are dominated by signs of hemolysis of red blood cells without reducing the hemoglobin index in the peripheral blood.According to world statistics, in the structure of morbidity among the pathologies of blood, the share of hemolytic states account for at least 5% of which are prevalent types of hereditary hemolytic anemia.
Symptoms of hemolytic anemia occur only when there is an obvious imbalance between the proliferation of blood cells erythrocyte number and the destruction of red blood cells in the circulating blood stream, while the compensatory function of the bone marrow (increased proliferation of reticulocytes) are depleted.
autoimmune hemolytic anemia
main precipitating factor in cau
Despite the rapid development of medicine in the field of diagnostic measures blood diseases, today it is not possible to establish reliable cause of autoimmune hemolytic anemia.
All clinical manifestations of autoimmune hemolytic anemia do not depend on the etiological factor.Most often, patients observed slow-progressive course of the disease.The first manifestations of the disease is a common weakness, aching joints, low-grade body temperature, pain in the abdomen.An objective examination of the patient a marked pallor and pasty skin, gradually increasing jaundice, enlargement of the spleen and liver.
In 50% of patients with an acute hemolytic anemia autoimmune nature, which is characterized by the sudden onset of the disease and stormy clinical picture.In this situation, to the fore numerous complaints of the patient in the absence of changes during physical examination of the patient.The main complaints that makes the patient are: severe weakness and decreased performance, increased heart rate, feeling short of breath, increase in body temperature to 38-39 degrees Celsius, headache and dizziness, nausea and vomiting not related to eating, aching pain in the girdleupper abdomen.External manifestations of hemolysis is only increasing yellowness of the skin without increasing the size of the liver and spleen.
signs of autoimmune hemolytic anemia in the nature of a laboratory study of blood are high reticulocytosis 200-300% decrease in the number of red blood cells in the normal color index, a slight leukocytosis, platelet count is not changed or slightly decreased.Absolute figures autommunnogo nature of anemia is to increase the ESR to high numbers.In some cases it is possible to identify microspherocytes or fragmented erythrocytes.At the biochemical study of blood is determined by increasing indirect bilirubin, hypergammaglobulinemia.To determine the presence of antibodies to red blood cells performed specific Coombs, which becomes sharply positive for autoimmune hemolytic anemia.
forecast for the life and safety performance in the autoimmune hemolytic anemia depends on the course, severity, and the effectiveness of the treatment of the underlying disease, which was the cause of hemolysis.As a rule, full recovery and disaster recovery can not be achieved if no one method of treatment.Sustained remission was observed only after radical splenectomy and a long course of hormone therapy.
Causes of hemolytic anemia
cause of hemolysis of red blood cells can serve as any physical illness and in such a situation develop acquired hemolytic anemia.
Under the influence of an etiological factor of developing clinical acute or chronic hemolytic state.
acute hemolytic anemia occurs most often when: a massive transfusion group incompatibility and Rh blood toxic effects of drugs of certain groups (nitrofurans, sulfonamides, quinidine, antipyretics), prolonged exposure to chemicals (benzene, lead), exposure to toxins atsnake bites, burns skin lesions, parasitic diseases.
Chronic hemolytic anemia observed in paroxysmal nocturnal hemoglobinuria, cold hemoglobinuria.
Hemolytic anemia symptoms
Classic symptoms of hemolytic anemia develops only when the intracellular hemolysis and presented anemic, jaundiced syndromes, and splenomegaly.
degree of clinical symptoms, evidence in favor of an anemic state, depends on the rate of destruction of red blood cells and bone marrow compensatory reaction, as the main hematopoietic organ.Symptoms of anemia develop only in a situation where the life of red blood cells is reduced to 15 days instead of 120 days.
Adrift distinguish latent (compensated), chronic (with severe anemizatsiey) and krizovoe hemolytic anemias.When crisis course of the disease is marked severe general condition of the patient, regardless of the type of hemolysis (intravascular or intracellular).
In the period of acute hemolytic anemia manifests itself as severe weakness, decreased performance, dizziness, shortness of breath, heart palpitations, drawing pains in the right and left hypochondria.A characteristic feature of acute hemolytic anemia is considered ikterichnost skin, mucous membrane of the soft palate and the sclera.An objective examination of the patient, in 70% of cases there is enlargement of the spleen and flush the liver out of the costal arch by more than 2 cm.
Due to condensation and stagnation of bile provoked the formation of stones in the gallbladder and bile ducts, so patients suffering from hemolytic anemia,often complain typical of hepatitis, cholecystitis and cholangitis (acute pain in the right upper quadrant, nausea, vomiting, fever, transient increase in body temperature).
The human body is constantly going on physiological erythrocyte hemolysis in the spleen.During the destruction of hemoglobin is released into the stream of indirect bilirubin circulating blood, which subsequently undergoes a change in the structure and composition of the liver, gall bladder and intestines.Withdrawal formed urobilin and stercobilin happens with urine and feces, so that patients often report dark urine and light stool.
In a situation where there is intravascular destruction of red blood cells to the fore the symptoms of anemia, while jaundice and splenomegaly are very rare.This type of hemolytic anemia characterized by a crisis course of the disease, largely worsen the patient's condition.The characteristic symptoms of hemolytic crisis are: general weakness, throbbing headache, feeling short of breath, heart palpitations, uncontrollable vomiting not related to eating, encircling aching pain in the upper abdomen and the squares of the lumbar region, fever, dark brown urine.In the absence of adequate therapy is rapidly developing clinical acute renal failure, which becomes the cause of death.
laboratory signs of hemolytic anemia include: normochromic, giperregeneratornost and change the shape and size of red blood cells (sickle-shaped, oval, microspherocytes, mishenevidnye).Characteristic changes in autoimmune hemolytic anemia is a leukocytosis with a shift to the left and leukocyte sharply increased ESR.When bone marrow puncture revealed hyperplasia of red sprout and expressed erythroblastic reaction.
Hemolytic anemia in children
In children, there are both congenital and acquired forms of hemolytic anemia.All hemolytic anemia have a common clinical symptoms, however, require the establishment of an accurate diagnosis indicating the forms of anemia, as it affects the further tactics of treatment and patient monitoring.
congenital hemolytic anemia, fortunately, is a rare disease and is not more than 2 cases per 100 000 population, but should be treated very carefully to children suffering from a form of anemia, as the treatment of these patients is extremely difficult.
hereditary hemolytic anemia Minkowski-Chauffard due to the manifestation of the defective gene, inherited in an autosomal dominant pattern, which causes changes in the shape of red blood cells.The shape changes as a result of violation of the cell membrane, and therefore, it becomes permeable to the penetration of an excessive amount of sodium ions, thus causing swelling of the erythrocyte.Excess haemolysis of red blood cell changes occur outside the splenic pulp.
onset of the disease occurs in early childhood and the first manifestations becomes ikterichnost mucous membranes and skin.Distinctive features of this form of hemolytic anemia is a combination of pronounced anemia syndrome with malformations (dolihotsefalicheskaya skull shape, saddle nose, high location of the hard palate).
main criteria for a diagnosis of "hereditary anemia Minkowski-Chauffard" are: normochromic, giperregeneratornost anemia, erythrocyte microspherocytosis, reduced osmotic resistance of red blood cells, increase in the indirect bilirubin in the blood, increasing the size of the spleen.
forecast for the life and health favorable for this form of anemia, occurs only after the radical splenectomy.
Another form of hereditary hemolytic anemia occurring in children, is anemia caused by lack of activity of G-6-PDG, which is characterized by an autosomal recessive mode of inheritance.
characteristic feature of this disease is the appearance of a spontaneous intravascular hemolysis after ingestion of certain groups of drugs (sulfonamides, quinine derivatives, antiplatelet agents, chloramphenicol, tubazid) or the use of legumes.
Clinical manifestations occur after 2-3 days after treatment and are manifested in the form of pronounced weakness, nausea and vomiting, rapid heartbeat, fever to febrile digits, anuria and acute renal failure phenomena.Distinctive features of this form of hereditary hemolytic anemia and hemoglobinuria considered gemosiderinuriya.
characteristic changes of peripheral blood are: decrease in the number of red blood cells and high reticulocytosis, presence of Heinz bodies in the red blood cell supravital staining, increased osmotic resistance of red blood cells.In the bone marrow punctate determined hyperplastic red germ hematopoiesis.
This type of anemia is characterized by a crisis course, so as a child needs preventive measures to completely eliminate from the diet of bean products, and to prevent the use of drugs at risk.Due to the fact that hereditary anemia often proceeds with the development of fulminant hemolytic crisis, accompanied by acute renal failure, the prognosis for the life and health of the child in this case is unfavorable.
most common form of hereditary hemolytic anemia in children is thalassemia, in which is contained in the erythrocyte pathologically excessive globin and accelerate aggregation and destruction of red blood cells not only in the spleen and bone marrow.
first manifestations of thalassemia occur in the neonatal period and manifest severe anemic syndrome, jaundice and splenomegaly in conjunction with abnormalities (square shape of the skull, protruding zygomatic arch, Mongoloid type of person, a flattening of the nose).In children suffering from thalassemia, lagging behind not only physically, but also psychomotor development.
thalassemia characteristic laboratory findings in children are: severe anemia (hemoglobin less reduction of 30 g / l), hypochromic (color index decline below 0.5), reticulocytosis, mishenevidnaya shape of red blood cells in the study of stroke, increased osmotic resistance of red blood cells, highthe level of indirect bilirubin.Absolute criteria for a diagnosis of "Thalassemia" is the elevated levels of fetal hemoglobin (30%).
Thalassemia is a constantly progressive pathology for which is not characteristic of a period of remission, in this connection, the level of mortality in this disease is very high.
Hemolytic anemia treatment
In order to maximize the positive results from the treatment of hemolytic anemia, you must first make every effort to clarify the authentic forms of the disease, since each treatment regimen should be Etiopatogenetichesky conditioned.
So, as a priority treatment of hereditary hemolytic anemia microspherocytic used radical splenectomy.Absolute indications for splenectomy are: krizovoe the disease with frequent periods of exacerbation, severe degree of anemia and hepatic colic.In most cases after splenectomy there is 100% remission of symptoms without recurrence of the disease despite the presence of microspherocytes blood.In situations where hemolysis accompanied by the appearance of stones in the gallbladder is recommended splenectomy combined with cholecystectomy.
as preventive measures recommended to patients receiving long choleretic (Allohol 1 tablet during each meal), antispasmodics (Riabal 1 capsule 2 p. / D) drugs, a periodical of duodenal intubation.In situations where there is a marked degree of anemia, it is advisable to replace the use of symptomatic red blood cell transfusions, taking into account group membership.
When the disease hereditary hemolytic anemia caused by a defect of G-6-PDG, recommended implementation of detoxification therapy (200 ml isotonic sodium chloride solution intravenously), as well as preventive measures for the prevention of disseminated intravascular coagulation (heparin 5000 U subcutaneously 4 p. / Day).
autoimmune hemolytic anemia responds well to therapy, hormonal therapy, which are used not only for the relief of hemolytic crisis, but also as a long-term use.To determine the optimal dosage of prednisolone is necessary to consider the overall health of the patient, as well as the peripheral blood.The adequate daily dose of prednisolone in this situation is considered 1 mg / kg body weight, but with no effect permissible increase in dose to 2.5 mg / kg.
In situations where autoimmune hemolytic anemia occurs in severe form, it is advisable to mix detoxification therapy (Neogemodez 200 ml intravenous-drip) from blood transfusions given group affiliation.Radical splenectomy is applied only in the absence of the effect of the use of conservative therapy and should be accompanied by the appointment of cytotoxic drugs (cyclophosphamide 100 mg 1 p. / D, azathioprine daily dose of 200 mg).