Efficacy and safety of allogeneic hematopoietic stem cell transplant in adults with hemophagocytic lymphohistiocytosis: a systematic review of literature

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.