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Acquired aplastic anemia
The disease occurs in all age groups; in the 0-17 age group, 1-2 cases per 1 million children are reported. The pathogenesis of acquired aplastic anemia (AAA) is based on immune-mediated depletion of the hematopoietic stem cell pool. In most cases, it is not possible to identify a specific etiologic factor that caused the development of the disease. In such cases, it is referred to as idiopathic (more than 70% of all cases) acquired aplastic anemia. Possible etiological factors include certain medications, exposure to chemical/toxic agents. Some viruses also play a certain role in the development of AAA: hepatitis A, B and C viruses, cytomegalovirus, parvovirus B19 and some others. AAA can develop as a complication of diseases associated with immune dysfunction.
The clinical manifestations of the disease may develop slowly and include anemic syndrome due to decreased red blood cell and hemoglobin levels, hemorrhagic syndrome caused by reduced platelet count and impaired function, and infectious complications due to a decrease in the content and total number of leukocytes. Therefore, the symptoms reported by patients to a doctor can be very diverse. Performing such a generally available laboratory test as a general blood test with a leukocyte count allows one to suspect aplastic anemia and refer the patient to a specialist. In some cases when the patient is not bothered by anything, and changes in the general blood test are detected during a preventive medical examination.
AAA must be differentiated from other conditions leading to pancytopenia, including inherited aplastic anemias (e.g., Fanconi anemia, Diamond-Blackfan anemia). A particular diagnostic challenge is distinguishing AAA from the hypoplastic variant of myelodysplastic syndrome.
Until recently, treatment of AAA was symptomatic. The one-year survival rate for patients receiving only symptomatic therapy did not exceed 10-20%. Death occurred from infectious complications or hemorrhages into vital structures (e.g., the brain).
Today, highly effective treatments for AAA are available. The optimal treatment for children is hematopoietic stem cell transplantation (HSCT or bone marrow transplantation) from a fully matched related donor, which is effective in 90% of cases. Unfortunately, fewer than 25% of patients have a compatible related donor.
For AAA patients without a matched related donor, first-line therapy consists of immunosuppressive therapy using anti-thymocyte globulin (rabbit or horse) and cyclosporine. The therapy is effective. According to various studies, its effectiveness is 55-85%. The patients who do not achieve a satisfactory hematologic response within a specified timeframe may receive a second course of immunosuppressive therapy using anti-thymocyte globulin from a different manufacturer or hematopoietic stem cell transplantation from an unrelated donor.
Diagnosis and treatment of AAA in children who are citizens of the Republic of Belarus is carried out only at the State Institution “Republican Scientific and Practical Center for Children’s Oncology, Hematology and Immunology”. Over a ten-year period from 2003 to 2012, AAA was diagnosed in the Republic of Belarus in 70 children aged 0-17. Treatment followed the principles described above. The overall survival rate of these patients was 81%.
Diagnosis and treatment of AAA in children who are citizens of the Republic of Belarus are performed at the State Institution “Republican Scientific and Practical Center for Children’s Oncology, Hematology and Immunology” regardless of their place of residence (registration) at the time of diagnosis. Diagnostics, treatment and subsequent monitoring in the consultative and outpatient department of the Center for citizens of the Republic of Belarus are free of charge. Patients who do not require intensive monitoring can undergo most of the treatment in the day care department, which makes the treatment more comfortable for the patient.
For foreign citizens, treatment is provided on a paid basis with the framework of the current legislation. The treatment costs of AAA include bed-day fees in the department, the cost of medications, blood products, laboratory reagents, consumables, examination of the donor of hematopoietic stem cells and the procedure for collecting cells.
The absence of a language barrier is an important advantage for citizens of CIS countries receiving treatment at the State Institution “Republican Scientific and Practical Center for Children’s Oncology, Hematology and Immunology”.
