title: Allogeneic hematopoietic stem-cell transplantation for adult and adolescent hemophagocytic lymphohistiocytosis: a single center analysis.
abstract: Myeloablative conditioning-based allogeneic hematopoietic stem-cell transplantation (allo-HSCT) in the treatment of adult and adolescent hemophagocytic lymphohistiocytosis (HLH) is rarely reported. We conducted a retrospective study of 30 adult and adolescent HLH transplanted for primary HLH (n = 4), tumor-HLH (n = 8), EBV-HLH (n = 14), and underlying disease-unknown (UDU)-HLH (n = 4). Peripheral blood stem cells (PBSCs) were the stem-cell source in all patients. Twenty-three patients were transplanted from HLA-haploidentical family donors, six from HLA-identical sibling donors, and one from a matched unrelated donor. Four patients appeared with mixed chimerism (MC), and no patient presented with graft failure. There was a high risk for EBV reactivation with an incidence of 47 %. Two patients developed post-transplant lymphoproliferative disorder (PTLD) and three were considered primary disease recurrent. With a median follow-up of 26 months, 19 patients survived and 11 patients died. The estimated 2-year overall survival (OS) was 63.3 ± 8.8 % in all patients, 100 % in primary HLH, 64.3 ± 12.8 % in EBV-HLH, 50.0 ± 17.7 % in tumor-HLH, and 50.0 ± 25.0 % in UDU-HLH. Myeloablative conditioning-based allo-HSCT is an effective treatment for adult and adolescent HLH to achieve complete remission and long-term survival.
authors.affiliation: Department of Hematology, Beijing Friendship Hospital, Capital Medical University, 95 Yong An Road, Xicheng District, Beijing, 100050, China.
authors.forename: Li
authors.initials: L
authors.lastname: Fu
authors.affiliation: Department of Hematology, Beijing Friendship Hospital, Capital Medical University, 95 Yong An Road, Xicheng District, Beijing, 100050, China.
authors.forename: Jingshi
authors.initials: J
authors.lastname: Wang
authors.affiliation: Department of Hematology, Beijing Friendship Hospital, Capital Medical University, 95 Yong An Road, Xicheng District, Beijing, 100050, China.
authors.forename: Na
authors.initials: N
authors.lastname: Wei
authors.affiliation: Department of Hematology, Beijing Friendship Hospital, Capital Medical University, 95 Yong An Road, Xicheng District, Beijing, 100050, China.
authors.forename: Lin
authors.initials: L
authors.lastname: Wu
authors.affiliation: Department of Hematology, Beijing Friendship Hospital, Capital Medical University, 95 Yong An Road, Xicheng District, Beijing, 100050, China.
authors.forename: Yini
authors.initials: Y
authors.lastname: Wang
authors.affiliation: Department of Hematology, Beijing Friendship Hospital, Capital Medical University, 95 Yong An Road, Xicheng District, Beijing, 100050, China.
authors.forename: Wenqiu
authors.initials: W
authors.lastname: Huang
authors.affiliation: Department of Hematology, Beijing Friendship Hospital, Capital Medical University, 95 Yong An Road, Xicheng District, Beijing, 100050, China.
authors.forename: Jia
authors.initials: J
authors.lastname: Zhang
authors.affiliation: Department of Hematology, Beijing Friendship Hospital, Capital Medical University, 95 Yong An Road, Xicheng District, Beijing, 100050, China.
authors.forename: Jinli
authors.initials: J
authors.lastname: Liu
authors.affiliation: Department of Hematology, Beijing Friendship Hospital, Capital Medical University, 95 Yong An Road, Xicheng District, Beijing, 100050, China. wangzhao1968@outlook.com.
authors.forename: Zhao
authors.initials: Z
authors.lastname: Wang
mesh_terms: D000293:Adolescent; D000328:Adult; D064591:Allografts; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects / Q000379:methods*; D004854:Herpesvirus 4, Human; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000209:etiology / Q000401:mortality / Q000628:therapy*; D008232:Lymphoproliferative Disorders / Q000209:etiology; D019653:Myeloablative Agonists / Q000627:therapeutic use; D009369:Neoplasms; D012008:Recurrence; D012074:Remission Induction / Q000379:methods; D012189:Retrospective Studies; D015996:Survival Rate; D014019:Tissue Donors; D019172:Transplantation Conditioning / Q000379:methods; D014775:Virus Activation
chemical_list: D019653:Myeloablative Agonists
keywords: Allogeneic hematopoietic stem-cell transplantation (allo-HSCT); Hemophagocytic lymphohistiocytosis (HLH); Myeloablative conditioning; Overall survival