医学
组织细胞
重症监护医学
病理生理学
鉴别诊断
移植
干细胞
生活质量(医疗保健)
免疫学
病理
儿科
内科学
生物
遗传学
护理部
作者
Manuel Ramos‐Casals,Pilar Brito‐Zerón,Armando López‐Guillermo,Munther A. Khamashta,Xavier Bosch
出处
期刊:The Lancet
[Elsevier]
日期:2013-11-27
卷期号:383 (9927): 1503-1516
被引量:1218
标识
DOI:10.1016/s0140-6736(13)61048-x
摘要
Haemophagocytic syndromes (haemophagocytic lymphohistiocytosis) have a wide range of causes, symptoms, and outcomes, but all lead to a hyperinflammatory response and organ damage—mainly reported in paediatric patients, but reports of adult presentation are increasing. Analysis of the genetic and molecular pathophysiology of these syndromes have improved the understanding of the crosstalk between lymphocytes and histiocytes and their regulatoty mechanisms. Clinical presentations with a broad differential diagnosis, and often life-threatening outcome, complicate the management, which might include supportive intensive care, immunosuppressive and biological treatments, or haemopoietic stem cell transplantation. Insufficient knowledge of these syndromes could contribute to poor prognosis. Early diagnosis is essential to initiate appropriate treatment and improve the quality of life and survival of patients with this challenging disorder.
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