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: | , , , , , |
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| Format: | Article |
| Language: | Russian |
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Journal Infectology
2019-10-01
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| 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. |
| format | Article |
| id | doaj-art-7c5c6596c36e4a04a5dff3d1e3795dc0 |
| institution | Matheson Library |
| issn | 2072-6732 |
| language | Russian |
| publishDate | 2019-10-01 |
| publisher | Journal Infectology |
| record_format | Article |
| series | Журнал инфектологии |
| spelling | doaj-art-7c5c6596c36e4a04a5dff3d1e3795dc02025-08-04T14:25:45ZrusJournal 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. Bykova5Pediatric Research and Clinical Center for Infectious DiseasesPediatric Research and Clinical Center for Infectious DiseasesPediatric Research and Clinical Center for Infectious DiseasesPediatric Research and Clinical Center for Infectious DiseasesResearch 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. PavlovResearch 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. PavlovFamily 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/943family hemophagocytic lymphogystyocytosishemophagocytic syndromeebv infection |
| 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 family hemophagocytic lymphogystyocytosis hemophagocytic syndrome ebv infection |
| 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 | family hemophagocytic lymphogystyocytosis hemophagocytic syndrome ebv infection |
| url | https://journal.niidi.ru/jofin/article/view/943 |
| work_keys_str_mv | AT naefremova familyhemophagocyticlymphohistiocytosis2clinicalcasesinonefamily AT lggoryacheva familyhemophagocyticlymphohistiocytosis2clinicalcasesinonefamily AT spkaplina familyhemophagocyticlymphohistiocytosis2clinicalcasesinonefamily AT vagreshnyakova familyhemophagocyticlymphohistiocytosis2clinicalcasesinonefamily AT aaosipova familyhemophagocyticlymphohistiocytosis2clinicalcasesinonefamily AT tabykova familyhemophagocyticlymphohistiocytosis2clinicalcasesinonefamily |