Chan K W, Mullen C A, Korbling M
Department of Pediatrics, Anderson Cancer Center, Houston, TX 77030, USA.
Bone Marrow Transplant. 1998 Aug;22(3):301-2. doi: 10.1038/sj.bmt.1701318.
Hemophagocytic lymphohistiocytosis (HLH) is a lethal disorder of immune dysregulation. Successful allogeneic BMT is the only therapy that has produced long-term disease-free survival. However, for patients with active HLH the outcome of BMT remains poor when compared with those transplanted in remission. We describe a patient with active, recurrent HLH treated with high-dose chemotherapy and related allogeneic peripheral blood stem cell (PBSC) transplantation. Hematologic recovery was rapid and the post-transplant course was uncomplicated. The patient is alive and well 2 1/2 years after transplant. Allogeneic PBSC transplantation may deserve further evaluation as an alternative source of stem cells for transplantation in patients with active HLH.
噬血细胞性淋巴组织细胞增生症（HLH）是一种免疫调节紊乱的致死性疾病。成功的异基因骨髓移植（BMT）是唯一能带来长期无病生存的治疗方法。然而，对于活动性HLH患者，与处于缓解期时接受移植的患者相比，BMT的结果仍然很差。我们描述了一名接受大剂量化疗和相关异基因外周血干细胞（PBSC）移植治疗的活动性、复发性HLH患者。血液学恢复迅速，移植后过程无并发症。该患者在移植后2年半时存活且状况良好。异基因PBSC移植作为活动性HLH患者移植干细胞的替代来源可能值得进一步评估。
