Family hemophagocytic lymphohistiocytosis: (2 clinical cases in one family)
https://doi.org/10.22625/2072-6732-2019-11-3-136-141
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.
About the Authors
N. A. Efremova
Pediatric Research and Clinical Center for Infectious Diseases
Russian Federation
Saint-Petersburg
Russian Federation
Saint-Petersburg
L. G. Goryacheva
Pediatric Research and Clinical Center for Infectious Diseases
Russian Federation
Saint-Petersburg
Russian Federation
Saint-Petersburg
S. P. Kaplina
Pediatric Research and Clinical Center for Infectious Diseases
Russian Federation
Saint-Petersburg
Russian Federation
Saint-Petersburg
V. A. Greshnyakova
Pediatric Research and Clinical Center for Infectious Diseases
Russian Federation
Saint-Petersburg
Russian Federation
Saint-Petersburg
A. A. Osipova
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
Russian Federation
Saint-Petersburg
Russian Federation
Saint-Petersburg
T. A. Bykova
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
Russian Federation
Saint-Petersburg
Russian Federation
Saint-Petersburg
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Review
For citations:
Efremova N.A., Goryacheva L.G., Kaplina S.P., Greshnyakova V.A., Osipova A.A., Bykova T.A. Family hemophagocytic lymphohistiocytosis: (2 clinical cases in one family). Journal Infectology. 2019;11(3):136-141. (In Russ.) https://doi.org/10.22625/2072-6732-2019-11-3-136-141
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