Аңдатпа
Background: Hairy cell leukemia is a rare oncohematological disease that manifests itself in damage to the bone marrow and spleen. One of the characteristic changes in the bone marrow are large cells, a rounded nucleus and thin outgrowths - “hairs” of the cytoplasm. The standard of care are drugs: cladribine and purine analogues-pentostatin. The complexity of therapy lies in the high risk of infections due to prolonged cytopenia during therapy. Aims: The aim of the study was to analyze the detection of hairy cell leukemia in Kazakhstan and the effectiveness of the cladribine regimen. Methods: In Kazakhstan 30 patients were diagnosed over the last 5-year period. All patients underwent cytological, histological examination of the bone marrow, bone marrow immunophenotyping. Thirty patient s were treated with cladribine intravenous infusion 0.1 mg/kg per day for 7 days. The average age was 54.6years (from 29 to 80 years), men-10, women-20. With a median follow-up of 4 years, the complete response (CR) was -25 patients, progression of disease – 3 patients, two patients die from infection. Results: Median duration of response was 38 months (range, 0.0+ to 52.0+). Median overall survival was not reached in all patients with CR. All patients had hematological complications in the form of grade 4 leukopenia - 30 patients, grade 4 thrombocytopenia - 28 patients, grade 3 thrombocytopenia – 2, patients grade 2 anemia - 9 patients. Emetic syndrome in the form of vomiting of the 3rd degree was in one patient, diarrhea of the 2nd degree in one patient. Eleven patients had febrile neutropenia. Two patients had a fatal outcome due to the development of pneumonia and subsequent sepsis. Summary/Conclusion: Treatment of patients with hairy cell leukemia with a purine analogue showed high efficiency, but was accompanied by prolonged cytopenia and frequent secondary infection. It is necessary to consider the issue of vaccination of this category of patients with anti-pneumococcal vaccine and also to conduct continuous monitoring. Ten patients with CR were vaccinated against coronavirus infection.