噬血细胞性淋巴组织细胞增多症
医学
造血干细胞移植
恶性肿瘤
儿科
化疗
干细胞
免疫学
移植
内科学
疾病
遗传学
生物
作者
Adeel Masood,Ahsan Wahab,Qamar Iqbal,James A. Davis,Hamid Ehsan,Hamza Hashmi
标识
DOI:10.1038/s41409-022-01668-9
摘要
Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening disorder of the immune system. While familial HLH is usually seen in children, secondary HLH is more common in adults. Secondary HLH is associated with a wide variety of underlying conditions including infections, malignancy and autoimmune disorders. While HLH 94/04 protocol-based chemotherapy can be used for initial treatment, allogeneic hematopoietic stem cell transplant (allo-HSCT) remains the only potentially curative treatment modality for this disorder. In this systematic review, we analyzed available literature on the role of allo-HSCT in adolescent and adult patients using PubMed, Cochrane, Embase and ClinicalTrials.gov. A total of 11 retrospective studies evaluated the role of allo-HSCT for HLH. Allo-HSCT, when compared to non-HSCT approach, appears to be associated with reasonable efficacy and acceptable safety for adolescent and adult patients with HLH.
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