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...
| Main Authors: | , , , , , |
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| Format: | Article |
| Language: | Russian |
| Published: |
Journal Infectology
2019-10-01
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| Series: | Журнал инфектологии |
| Subjects: | |
| Online Access: | https://journal.niidi.ru/jofin/article/view/943 |
| Summary: | 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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| ISSN: | 2072-6732 |