The clinical characteristics of adult hemophagocytic lymphohistiocytosis treated with haploidentical donor hematopoietic stem cell transplantation / 中华内科杂志

ABSTRACT

Objective To analyze the clinical characteristics of adult patients with hemophagocytic lymphohistiocytosis (HLH) receiving haploidentical donor hematopoietic stem cell transplantation (HID HSCT).Method We retrospectively reviewed 20 adult patients with HLH from August 2009 to August 2014.The clinical features and outcome were analyzed.Results Conditioning regimens consisted of total body irradiation/etoposide/cyclophosphamide (TBI/VP-16/CTX) and busulfan (Bu)/VP-16/CTX in HLH with anti-thymocyte globulin (ATG) 8 mg/kg.The stem cells were mobilized from donors' peripheral blood.Median time to white blood cell engraftment was 13 (9-27) days.Median time to platelet engraftment was 14 (10-28) days.Mixed chimerism after transplantation developed in 4 patients and no patient presented graft failure.Eight patients developed grade Ⅱ to Ⅲ acute graft-versus-host disease (GVHD),while as chronic GVHD occurred in 9 patients.Among 12 patients with EB virus (EBV) reactivation,2 patients developed post-transplant lymphoproliferative disorder (PTLD),7 were suspected as PTLD and 3 were considered as relapse of primary disease.With a median follow-up of 20 months (range0.5-108 months) after transplantation,the estimated 2-year overall survival (OS) rate was (60.0 ± 11.0) ％ in all patients.During the follow-up,12 patients survived,8 died including 5 within 100 days after HSCT.Among 5 non-remission patients before HSCT,4 patients died within 100 days after HCT.Conclusions HID HSCT is an effective treatment for adult patients with HLH to achieve remission and long-term survival.High proportion of mixed chimerism has been seen at early stage after transplantation.EBV reactivation and early transplant-related mortality are common.