- a group of diseases associated with a decrease in the length of the bed of circulating red blood cells of blood because of their destruction, or hemolysis.They constitute more than 11% of all cases of anemia and more than 5% of all hematologic diseases.
This article will talk about the causes of this illness and the treatment of this complex disease.
Red blood cells or red blood cells - red blood cells is the main function of which is to transport to the organs and tissues of oxygen.Shaped red blood cells in the bone marrow, where they mature forms go into the bloodstream and circulate throu
Depending on the nature of the flow, hemolytic anemia, there are acute and chronic.
depending on the causative agent disease can be congenital (hereditary) or acquired:
1. Hereditary hemolytic anemia:
2. Acquired hemolytic anemia:
pathogenesis of hemolytic anemia in different variants of the disease varies.In general, it can be represented as follows.Destroys red blood cells can be in two ways: intravascular and intracellular.Reinforced lysis of them inside the vessel is often caused by mechanical damage, exposure of cells to toxins caught outside, fixation on the surface of red blood cell immune cells.
extravascular hemolysis is performed in the spleen and liver.Amplifies it in the event of changes in the properties of erythrocyte membranes (for example, if there are fixed immunoglobulins), as well as limiting the possibility of red blood cells to change shape (this dramatically complicates their normal passage through the vessels of the spleen).In various forms of hemolytic anemia, these factors combine to varying degrees.
Clinical signs of the disease are hemolytic syndrome, and, in severe cases, hemolytic crisis.
clinical and hematological manifestations of hemolytic syndrome different for intravascular hemolysis and intracellular different.
Signs of intravascular hemolysis:
Intracellular hemolysis is characterized by the following features:
hemolytic crisis - a state of massive hemolysis of red blood cells, characterized by the sharp deterioration of the general condition of the patient, the progression of acute anemia.It requires immediate hospitalization and emergency treatment begins.
The first efforts of doctors in the treatment of this disease should be aimed at addressing the causes of hemolysis.Parallel conduct pathogenetic therapy, as a rule, the use of drugs, immunosuppressive agents which depress the immune system, replacement therapy (transfusion of blood components, in particular, canned red blood cells), detoxification (infusion of saline, reopoliglyukina and so on), and attempt to eliminate unpleasant for the patient's symptomsdisease.
Let us consider some clinical forms of hemolytic anemia.
In this disease increases the permeability of the membrane of erythrocytes, they penetrate sodium ions.Type Inheritance - autosomal dominant.The first symptoms appear, usually in childhood or adolescence.
occur in waves, periods of stability suddenly replaced hemolytic crisis.
characterized by the following triad of symptoms:
Based on the data above, we can say that in the blood is determined by anemia: normo- or microcytic, giperregeneratornaya.
clinically manifested by mild jaundice (in the blood while increasing the level of indirect bilirubin), an enlarged spleen and liver.Frequent so-called stigma disembriogeneza - "tower skull", uneven tooth alignment, adherent earlobe, slanted eyes, and so on.
Treatment with mild anemia Minkovsky-Shofar is not carried out.In the case of heavy flow it to the patient removes the spleen - splenectomy.
It is a whole group of diseases transmitted by inheritance, arising from violation of the synthesis of one or more chains of hemoglobin.It may be homo- or heterozygous.Typically, most disturbed forming one of the chains of hemoglobin, and the second is produced in normal amounts, but because of its greater excess precipitates.Suspected
talessemiyu help following features:
confirms the diagnosis of the disease in the presence of one or more close relatives.
treatment is carried out in periods of exacerbation: the patient is prescribed a transfusion of red blood cells preserved and vitamin B9 (folic acid).If significantly increased spleen, splenectomy is performed.
This form is the most common hemoglobin disorders.Suffer it usually faces blacks.It is characterized by the presence of a disease in a patient specific type of hemoglobin - hemoglobin S, which is one in a chain of amino acids - glutamine - replaced by another - valine.Due to this nuance hemoglobin S 100 times less soluble than hemoglobin A developing camber phenomenon, erythrocytes acquire specific shape - the shape of a sickle, becoming less compliant - do not change their shape, because of which easily get stuck in the capillaries.Clinically it is manifested by frequent thrombosis in different organs: patients complain of pain and swelling of the joints, intense abdominal pain, they myocardial lung and spleen.
can develop hemolytic crisis, manifested release black, painted in blood, urine, a sharp decrease in the level of hemoglobin in the blood fever.Outside
crisis in the blood of the patient is determined by the anemia of moderate severity with the presence of red blood cells in a smear crescent-shaped, reticulocytosis.Also elevated levels of bilirubin in the blood.The large amount of bone marrow erythrokaryocytes.
sickle cell disease is difficult to control.A patient in need of administering to the bulk fluid, as a result, reduces the amount of modified erythrocytes and decreases the risk of thrombosis.Parallel conduct oxygen therapy and antibiotics (to fight infectious complications).In severe cases, the patient shows red blood cell transfusions and even splenectomy.
This form of hereditary hemolytic anemia associated with impaired synthesis of porphyrin - natural pigments that make up hemoglobin.Sent to the X chromosome it occurs, usually boys.
first symptoms appear in childhood: is hypochromic anemia, progressing over the years.Over time, there are signs of deposits in the organs and tissues of iron - hemosiderosis:
in the case of iron accumulation in the pancreas develops insulin deficiency: diabetes.Erythrocytes
acquire mishenevidnuyu shape are of different sizes and shapes.The level of iron in serum is 2-3 times higher than the normal values.Transferrin saturation approaches 100%.In the bone marrow sideroblasts are determined, and in erythrokaryocytes around their cores disposed iron nuggets.
possible option and acquired porphyria.As a rule, he is diagnosed with lead poisoning.Clinically it is manifested signs of damage to the nervous system (encephalitis, polyneuritis), digestive tract (lead colic), skin (pale with an earthy hue).On the gums appear specific lead border.It confirms the diagnosis by examining the level of lead in the urine of the patient: in this case it will be upgraded.
In the case of acquired forms of porphyria therapeutic interventions should be directed to treatment of the underlying disease.Patients with hereditary forms of its canned transfusions of red blood cells.A radical method of treatment is surgery to bone marrow transplantation.
an acute attack of porphyria If a patient is administered glucose and hematin.To prevent hemochromatosis, phlebotomy spend up to 300-500 ml of 1 times a week to reduce the hemoglobin to 110-120 g / L or until remission.
disease characterized by destruction of red blood cells increased antibodies to the antigens of their membranes or sensitive to them lymphocytes.Often the primary or secondary (symptomatic).Recent met several times more primary and accompany some other diseases - cirrhosis of the liver, hepatitis, infection, immunodeficiency states.
in the diagnosis of this type of anemia should pay special attention to the history and comprehensive survey, only reliably be known as the true cause of anemia.Also conducts direct and indirect Coombs' test.
The leading role belongs to the treatment of addressing the underlying causes of the disease along with the appointment of steroid hormones: prednisolone, dexamethasone.If the patient's reaction to the introduction of the hormones missing, conducted splenectomy and appoint cytostatics.