title: [Successful treatment of agammaglobulinemia by HLA-mismatched unrelated cord blood stem cell transplantation--the first case report].
abstract: OBJECTIVE
  To evaluate cord blood stem cell transplantation (CBT) in the treatment of X-linked agammaglobulinemia, and observe the courses of the hematopoietic and immune reconstitution.
  
  
  METHODS
  A 14-year-old male patient with agammaglobulinemia received CBT from a 1/6 HLA-mismatched unrelated cord blood. The conditioning regimen was Bu/Cy/anti-CD3 antibody. CsA was given together with MMF and MTX for prophylaxis of GVHD. The patient received 0.42 x 10(8) nucleated cells/kg, containing 0.35 x 10(6) CD34(+) cells/kg.
  
  
  RESULTS
  The recipient showed hematopoietic reconstitution on day 30 post-transplantation when ANC was 0.5 x 10(9)/L and BPC 20 x 10(9)/L. Sex chromosome analysis showed engraftment (donor 46, XX/recipient 46, XY = 4:1) on day 45. The recipient's blood group changed from AB to O, IgG from 1.1 g/L to 3.5 g/L, sex chromosome from 46, XY to full 46, XX, and mature B cells in peripheral blood from 0 to 5% on day 100, indicating immune reconstitution. At the last follow-up of 360 days, the patient without acute or chronic GVHD showed normal hemogram and myelogram, IgG 13.5 g/L and 10% mature B cells in peripheral blood, indicating the hematopoiesis and immune persistent reconstitution. No acute or chronic GVHD was developed.
  
  
  CONCLUSIONS
  This is the first case report of successful treatment of X-linked agammaglobulinemia by HLA-mismatched unrelated CBT.
authors.affiliation: Department of Hematology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China.
authors.forename: Ding-ming
authors.initials: DM
authors.lastname: Wan
authors.forename: Chang-feng
authors.initials: CF
authors.lastname: Liu
authors.forename: Gui-ju
authors.initials: GJ
authors.lastname: Wang
authors.forename: Hui
authors.initials: H
authors.lastname: Sun
authors.forename: Ling
authors.initials: L
authors.lastname: Sun
authors.forename: Zhong-xing
authors.initials: ZX
authors.lastname: Jiang
authors.forename: Wan-li
authors.initials: WL
authors.lastname: Guo
authors.forename: Ling
authors.initials: L
authors.lastname: Qin
authors.forename: Shao-jun
authors.initials: SJ
authors.lastname: Liu
mesh_terms: D000293:Adolescent; D000361:Agammaglobulinemia / Q000601:surgery*; D036101:Cord Blood Stem Cell Transplantation / Q000379:methods*; D006086:Graft vs Host Disease / Q000517:prevention & control; D006680:HLA Antigens / Q000276:immunology; D006801:Humans; D008297:Male; D019172:Transplantation Conditioning; D016896:Treatment Outcome
chemical_list: D006680:HLA Antigens