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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Format: | Article |
Language: | Russian |
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Journal Infectology
2019-10-01
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Series: | Журнал инфектологии |
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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. |
first_indexed | 2024-03-12T03:09:21Z |
format | Article |
id | doaj.art-7c5c6596c36e4a04a5dff3d1e3795dc0 |
institution | Directory Open Access Journal |
issn | 2072-6732 |
language | Russian |
last_indexed | 2024-03-12T03:09:21Z |
publishDate | 2019-10-01 |
publisher | Journal Infectology |
record_format | Article |
series | Журнал инфектологии |
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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