Academic Journal
Hemophagocytic lymphohistiocytosis and macrophage activation syndrome: two rare sides of the same devastating coin
| العنوان: | Hemophagocytic lymphohistiocytosis and macrophage activation syndrome: two rare sides of the same devastating coin |
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| المؤلفون: | Flavio Sztajnbok, Adriana Rodrigues Fonseca, Leonardo Rodrigues Campos, Kátia Lino, Marta Cristine Félix Rodrigues, Rodrigo Moulin Silva, Rozana Gasparello de Almeida, Sandro Félix Perazzio, Margarida de Fátima Fernandes Carvalho |
| المصدر: | Advances in Rheumatology, Vol 64, Iss 1, Pp 1-11 (2024) |
| بيانات النشر: | BMC, 2024. |
| سنة النشر: | 2024 |
| المجموعة: | LCC:Diseases of the musculoskeletal system LCC:Immunologic diseases. Allergy |
| مصطلحات موضوعية: | Macrophage activation syndrome, Hemophagocytic lymphohistiocytosis, Cytokines, Juvenile idiopathic arthritis, Diseases of the musculoskeletal system, RC925-935, Immunologic diseases. Allergy, RC581-607 |
| الوصف: | Abstract Hemophagocytic lymphohistiocytosis (HLH) is a rare genetic hyperinflammatory syndrome that occurs early in life. Macrophage activation syndrome (MAS) usually refers to a secondary form of HLH associated with autoimmunity, although there are other causes of secondary HLH, such as infections and malignancy. In this article, we reviewed the concepts, epidemiology, clinical and laboratory features, diagnosis, differential diagnosis, prognosis, and treatment of HLH and MAS. We also reviewed the presence of MAS in the most common autoimmune diseases that affect children. Both are severe diseases that require prompt diagnosis and treatment to avoid morbidity and mortality. |
| نوع الوثيقة: | article |
| وصف الملف: | electronic resource |
| اللغة: | English |
| تدمد: | 2523-3106 |
| Relation: | https://doaj.org/toc/2523-3106 |
| DOI: | 10.1186/s42358-024-00370-2 |
| URL الوصول: | https://doaj.org/article/df8667ebfca942c4920033bc5f49547e |
| رقم الانضمام: | edsdoj.f8667ebfca942c4920033bc5f49547e |
| قاعدة البيانات: | Directory of Open Access Journals |
| تدمد: | 25233106 |
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| DOI: | 10.1186/s42358-024-00370-2 |