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

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 includ...

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Main Authors: N. A. Efremova, L. G. Goryacheva, S. P. Kaplina, V. A. Greshnyakova, A. A. Osipova, T. A. Bykova
Format: Article
Language:Russian
Published: Journal Infectology 2019-10-01
Series:Журнал инфектологии
Subjects:
Online Access:https://journal.niidi.ru/jofin/article/view/943
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author N. A. Efremova
L. G. Goryacheva
S. P. Kaplina
V. A. Greshnyakova
A. A. Osipova
T. A. Bykova
author_facet N. A. Efremova
L. G. Goryacheva
S. P. Kaplina
V. A. Greshnyakova
A. A. Osipova
T. A. Bykova
author_sort N. A. Efremova
collection DOAJ
description 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.
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spelling doaj.art-7c5c6596c36e4a04a5dff3d1e3795dc02023-09-03T14:29:58ZrusJournal InfectologyЖурнал инфектологии2072-67322019-10-0111313614110.22625/2072-6732-2019-11-3-136-141751Family hemophagocytic lymphohistiocytosis: (2 clinical cases in one family)N. A. Efremova0L. G. Goryacheva1S. P. Kaplina2V. A. Greshnyakova3A. A. Osipova4T. A. Bykova5Детский научно-клинический центр инфекционных болезнейДетский научно-клинический центр инфекционных болезнейДетский научно-клинический центр инфекционных болезнейДетский научно-клинический центр инфекционных болезнейНаучно-исследовательский институт детской онкологии, гематологии и трансплантологии им. Р.М. Горбачевой Первого Санкт- Петербургского государственного медицинского университета им. академика И.П. ПавловаНаучно-исследовательский институт детской онкологии, гематологии и трансплантологии им. Р.М. Горбачевой Первого Санкт- Петербургского государственного медицинского университета им. академика И.П. ПавловаFamily 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.https://journal.niidi.ru/jofin/article/view/943семейный гемофагоцитарный лимфогистиоцитозгемофагоцитарный синдромebvинфекция
spellingShingle N. A. Efremova
L. G. Goryacheva
S. P. Kaplina
V. A. Greshnyakova
A. A. Osipova
T. A. Bykova
Family hemophagocytic lymphohistiocytosis: (2 clinical cases in one family)
Журнал инфектологии
семейный гемофагоцитарный лимфогистиоцитоз
гемофагоцитарный синдром
ebvинфекция
title Family hemophagocytic lymphohistiocytosis: (2 clinical cases in one family)
title_full Family hemophagocytic lymphohistiocytosis: (2 clinical cases in one family)
title_fullStr Family hemophagocytic lymphohistiocytosis: (2 clinical cases in one family)
title_full_unstemmed Family hemophagocytic lymphohistiocytosis: (2 clinical cases in one family)
title_short Family hemophagocytic lymphohistiocytosis: (2 clinical cases in one family)
title_sort family hemophagocytic lymphohistiocytosis 2 clinical cases in one family
topic семейный гемофагоцитарный лимфогистиоцитоз
гемофагоцитарный синдром
ebvинфекция
url https://journal.niidi.ru/jofin/article/view/943
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AT vagreshnyakova familyhemophagocyticlymphohistiocytosis2clinicalcasesinonefamily
AT aaosipova familyhemophagocyticlymphohistiocytosis2clinicalcasesinonefamily
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