Adult haemophagocytic lymphohistiocytosis: a review
| Autor: | Etienne Danse, Jean Cyr Yombi, Van Den Neste E, Halil Yildiz, Jean-Philippe Defour |
|---|---|
| Přispěvatelé: | UCL - SSS/IREC/SLUC - Pôle St.-Luc, UCL - SSS/DDUV/BCHM - Biochimie-Recherche métabolique, UCL - SSS/DDUV/SIGN - Cell signalling, UCL - SSS/IREC/IMAG - Pôle d'imagerie médicale, UCL - (SLuc) Service de radiologie, UCL - (SLuc) Service de biologie hématologique, UCL - (SLuc) Service d'hématologie, UCL - (SLuc) Service de médecine interne générale, UCL - (SLuc) Centre de prise en charge (H.I.V.), UCL - (SLuc) Centre du cancer |
| Rok vydání: | 2020 |
| Předmět: |
Ruxolitinib
Pediatrics medicine.medical_specialty ruxolitinib macrophagic activation syndrome autoimmune disease Disease Malignancy 03 medical and health sciences 0302 clinical medicine alemtuzumab emapalumab Medicine 030212 general & internal medicine Etoposide Autoimmune disease Hemophagocytic lymphohistiocytosis business.industry Mortality rate General Medicine medicine.disease infection hemophagocytic lymphohistiocytosis 030220 oncology & carcinogenesis Alemtuzumab genetic business malignancy medicine.drug |
| Zdroj: | QJM, Vol. 115, no. 4, p. 205-213 (2022) |
| ISSN: | 1460-2393 1460-2725 |
| DOI: | 10.1093/qjmed/hcaa011 |
| Popis: | Haemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder characterized by hyperimmune response. The mortality is high despite progress being made in the diagnosis and treatment of the disease. This review aimed to update knowledge on adult HLH pathophysiology, identify the numerous causes and help clinicians make early diagnosis and initiate treatment. Using Embase, we searched relevant articles published from 1 January 2010 to 31 October 2019, with the MESH term ‘haemophagocytic lymphohistiocytosis; macrophagic activation syndrome, adult’. The mean age at presentation is about 50 years, with a male predominance. The most frequent disease associations are haematological diseases, viral or bacterial infections and autoimmune diseases. The pathophysiologic mechanism is probably the combination of inherited genetic mutations and extrinsic triggers. The mortality rate is 26.5–74.8%. HScore is more efficient than HLH-2004 criteria to identify HLH, with diagnostic sensitivity and specificity 90% and 79%, respectively. 18-fluorodeoxyglucose positron emission tomography–computed tomography is potentially useful for detecting underlying disease and the extent of secondary HLH. Disease-specific treatment should be given as soon as possible. Treatment with corticosteroids combined or not with etoposide is the mainstay of treatment. Monoclonal antibodies and JAK pathway inhibitors show promise of being effective. In adult HLH, infectious diseases, autoimmune disease and malignancy should be suspected so that disease-specific treatment can be given promptly. Treatment with corticosteroids combined or not with etoposide is the mainstay of treatment, but new therapies show promise of being effective. |
| Databáze: | OpenAIRE |
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