title: [The clinical characteristics of adult hemophagocytic lymphohistiocytosis treated with haploidentical donor hematopoietic stem cell transplantation].
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 (range: 0.5-108 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.
authors.affiliation: Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China.
authors.forename: L
authors.initials: L
authors.lastname: Fu
authors.forename: N
authors.initials: N
authors.lastname: Wei
authors.forename: J S
authors.initials: JS
authors.lastname: Wang
authors.forename: L
authors.initials: L
authors.lastname: Wu
authors.forename: Y N
authors.initials: YN
authors.lastname: Wang
authors.forename: D Y
authors.initials: DY
authors.lastname: Huang
authors.forename: J L
authors.initials: JL
authors.lastname: Liu
authors.forename: Z
authors.initials: Z
authors.lastname: Wang
mesh_terms: D000293:Adolescent; D000328:Adult; D000961:Antilymphocyte Serum / Q000008:administration & dosage / Q000627:therapeutic use*; D018906:Antineoplastic Agents, Alkylating / Q000008:administration & dosage / Q000627:therapeutic use*; D002066:Busulfan / Q000008:administration & dosage / Q000627:therapeutic use*; D046528:Chimerism; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000627:therapeutic use*; D020031:Epstein-Barr Virus Infections / Q000209:etiology; D005260:Female; D006086:Graft vs Host Disease; D018380:Hematopoietic Stem Cell Transplantation / Q000379:methods*; D004854:Herpesvirus 4, Human; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000401:mortality / Q000628:therapy*; D008297:Male; D011184:Postoperative Period; D012189:Retrospective Studies; D015996:Survival Rate; D014019:Tissue Donors; D019172:Transplantation Conditioning / Q000379:methods*; D014184:Transplantation, Homologous / Q000379:methods*; D016896:Treatment Outcome; D014916:Whole-Body Irradiation / Q000009:adverse effects* / Q000379:methods
chemical_list: D000961:Antilymphocyte Serum; D018906:Antineoplastic Agents, Alkylating; D003520:Cyclophosphamide; D002066:Busulfan
keywords: Haploidentical donor; Hematopoietic stem cell transplantation; Lymphohistiocytosis, hemophagocytic; Mixed chimerism