Symptoms and tactics of treatment of aplastic anemia

Hypo- or aplastic anemia is a group of pathological conditions in which bone marrow blood formation is inhibited with the development of hypo- or aplasia of the bone marrow in the absence of signs of hemoblastoses. In this case, the hematopoietic tissue is replaced by adipose tissue or connective tissue. The incidence is 3-6 cases per 1 million population. Read the causes, mechanism of development, clinical manifestations and treatment of aplastic anemia in our article.

Causes and mechanism of development of aplastic anemia

Depending on the cells that are the precursors of hematopoiesis, aplastic anemia can be single- or multi-germ (pancytopenia). Depression of hematopoiesis can be acquired and congenital.

In aplastic anemia, several pathogenetic moments are distinguished. Of primary importance is the reduction in the content of stem cells and the presence of an internal defect in them, which is further manifested by the inhibition of all the main germs of hematopoiesis. It is believed that the immunosuppressive effect on early cells - precursors of hematopoiesis plays an important role.

Main causes of aplastic anemia

1. Congenital:

• Schwachman-Diamond syndrome;
• Fanconi anemia;
• congenital dyskeratosis;
• reticular dysgenesis;
• familial aplastic anemia;
• non-hematological syndromes (Dine syndrome).

2. Acquired Causes:

• idiopathic aplastic anemia – accounts for 80% of cases;
• infections (hepatitis, cytomegalovirus, HIV, parvovirus, Epstein-Barr virus);
• toxic effect of chemicals, ionizing radiation;
• toxic effect of drugs (mercasolil, sulfonamides, cytostatics, antibiotics, NSAIDs, antiepileptic drugs);
• immunological disorders (eosinophilic fasciitis, thymoma, SLE, RA, rejection reaction);
• pregnancy.

Clinical picture of the development of aplastic anemia

The disease sometimes begins acutely and progresses at lightning speed, but more often it develops gradually. In the clinical picture of aplastic anemia, 3 syndromes can be distinguished:

• anemic;
• hemorrhagic;
• infectious - toxic (immunodeficiency).

Anemic syndrome is manifested by the development of tachycardia, shortness of breath, the presence of systolic murmur over the area of ​​the heart, pallor of the skin. Infectious - toxic develops against the background of severe granulocytopenia. At the same time, fever and other infectious and toxic complications are present - tonsillitis, otitis media, pneumonia, respiratory tract infections.

Due to thrombocytopenia, hemorrhagic syndrome develops – petechiae, ecchymosis appear on the skin, uterine and nosebleeds develop. Blood tests show pancytopenia and elevated erythrocyte sedimentation rate. Crucial in diagnosing aplastic anemia is sternal puncture (morphological examination) and trepanobiopsy (histological examination). They reveal the inhibition of all hematopoietic germs and the replacement of hematopoietic tissue with adipose tissue.

What are the aspects of aplastic anemia treatment?

Therapy is prescribed depending on the form of aplastic anemia. With mild forms, a known etiological factor, as well as with information about the gradual resumption of hematopoiesis, the most important is the regime that prevents the occurrence of infectious complications. Of the drugs, anabolic drugs are recommended.

In severe forms of aplastic anemia, when the number of reticulocytes in the blood is less than 0.5x10⁹/l, and platelets are less than 20x10⁹/l, the main treatment is allogeneic bone marrow transplantation which is histologically compatible. Timely transplantation cures 85% of patients, especially those under the age of 30.

If allogeneic bone marrow transplantation is not possible, the only treatment for aplastic anemia is long-term immunosuppressive treatment (immunoablation). Such treatment should begin as soon as possible. Anti-lymphocyte immunoglobulin, prednisolone and cyclosporine A are prescribed.

In the case of severe granulocytopenia, it is necessary to provide the patient with aseptic conditions (boxing chambers, isolation from others, decontamination of the intestine), prescribe broad-spectrum antibiotics. With severe anemia, an erythrocyte mass is used, with thrombocytopenia and hemorrhagic syndrome – platelet concentrate.