Family hemophagocytic lymphohistiocytosis: (2 clinical cases in one family)-Reference-Cited by-同舟云学术

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

Published:2019-10-09 Issue:3 Volume:11 Page:136-141
ISSN:2499-9865
Container-title:Journal Infectology
language:
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Author:

Efremova N. A.¹,Goryacheva L. G.¹,Kaplina S. P.¹,Greshnyakova V. A.¹,Osipova A. A.²,Bykova T. A.²

Affiliation:

1. Pediatric Research and Clinical Center for Infectious Diseases

2. Research Institute of Children Oncology, Hematology and Transplantology named after R.M. Gorbacheva of The First Saint-Petersburg State Medical University named after academician I.P. Pavlov

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.

Publisher

SPRIDA

Subject

Infectious Diseases

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