Family hemophagocytic lymphohistiocytosis: (2 clinical cases in one family)

Abstract

Family hemophagocytic lymphohistiocytosis (hemophagocytic syndrome) is a rare hereditary disease, which is based on a disturbance of the regulation of the immune response, leading to proliferation and activation of histiocytes, phagocytosis of peripheral blood cells. The most common mutations include – PRF1, UNC13D, STX11. Two cases of familial hemophagocytic lymphogystyocytosis in children of an early age from a single family, features of the course are described.

Keywords

• family hemophagocytic lymphogystyocytosis
• hemophagocytic syndrome
• ebv infection