title: Hematopoietic Stem Cell Transplant for Primary Immunodeficiency Diseases: A Single-Center Experience.
abstract: OBJECTIVE
  The only curative treatment for many patients with primary immunodeficiency disease is hematopoietic stem cell transplant. In this study, we report the transplant outcomes of patients with primary immunodeficiency diseases.
  
  
  METHODS
  Herein, we present the transplant outcomes of 20 patients with primary immunodeficiency disease seen at our center in Kayseri, Turkey, from 2010 to 2015.
  
  
  RESULTS
  The disease distribution of the 20 patients were as follows: 6 patients with severe combined immunodeficiency, 4 patients with hemophagocytic lymphohistiocytosis, 2 patients with chronic granulomatous disease, 2 patients with type 2 Griscelli syndrome, 2 patients with B-cell deficiency plus bone marrow failure, 1 patient with severe congenital neutropenia, 1 patient with X-linked lymphoproliferative disease, 1 patient with T-cell deficiency plus relapsed non-Hodgkin lymphoma, and 1 patient with type 1 leukocyte adhesion deficiency. Of the 20 patients, 11 received related HLA-matched, 6 received haploidentical, 2 received unrelated HLA-matched, and 1 received HLA-mismatched transplant. The median age at transplant was 21 months, and median follow-up was 5 months. Overall survival rate was 65%. Mean engraftment times for neutrophils and platelets were 14.25 ± 3.08 and 24.7 ± 11.4 days. Graft-versus-host disease was observed in 30% of patients.
  
  
  CONCLUSIONS
  Patients with primary immunodeficiency disease treated at our center had acceptable transplant outcomes. This study supports the use of hematopoietic stem cell transplant in patients with primary immunodeficiency disease.
authors.affiliation: From the Department of Pediatric Immunology, and the Department of Pediatric Hematology and Oncology, Erciyes University School of Medicine, Kayseri, Turkey.
authors.forename: Turkan
authors.initials: T
authors.lastname: Patiroglu
authors.forename: H Haluk
authors.initials: HH
authors.lastname: Akar
authors.forename: Ekrem
authors.initials: E
authors.lastname: Unal
authors.forename: M Akif
authors.initials: MA
authors.lastname: Ozdemir
authors.forename: Musa
authors.initials: M
authors.lastname: Karakukcu
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003141:Communicable Diseases / Q000209:etiology; D005260:Female; D006086:Graft vs Host Disease / Q000209:etiology; D006680:HLA Antigens / Q000276:immunology; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects; D006648:Histocompatibility; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000175:diagnosis / Q000276:immunology / Q000601:surgery*; D007223:Infant; D008297:Male; D012307:Risk Factors; D013997:Time Factors; D000075442:Transplantation, Haploidentical; D016896:Treatment Outcome; D014421:Turkey
chemical_list: D006680:HLA Antigens