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HEMOPHAGOCYTAL LYMPHOHISTIOCYTOSIS

Abstract

Hemophagocytal lymphohistiocytosis is a rare and frequently fatal disorder of immune response regulation, based on defects of cell cytotoxicity and hyper production of pro inflammatory cytokines. This disease is devided to genetically determinate types of hemophagocytal lymphohistiocytosis and secondary hemophagocytal syndromes, associated to infections, malignant neoplasm, autoimmune disease and immunodeficiency. The article gives a brief description of pathogenesis, clinical symptoms, and methods of hemophagocytal lymphohistiocytosis treatment.
Key words: hemophagocytal lymphohistiocytosis, immunosuppression, ciclosporin A, etoposide, dexamethasone.
(Voprosy sovremennoi pediatrii — Current Pediatrics. 2009;8(3):66-75)

About the Authors

M.A. Maschan
Federal Scientific Clinical Center of Children's Hematology, Oncology, and Immunology, Moscow Russian Children's Clinical Hospital, Moscow
Russian Federation



G.A. Novichkova
Federal Scientific Clinical Center of Children's Hematology, Oncology, and Immunology, Moscow
Russian Federation


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For citations:


Maschan M., Novichkova G. HEMOPHAGOCYTAL LYMPHOHISTIOCYTOSIS. Current Pediatrics. 2009;8(3):66-75.

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