Mismatched related vs matched unrelated donors in TCRαβ/CD19-depleted HSCT for primary immunodeficiencies.

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  • Additional Information
    • Source:
      Publisher: Elsevier Country of Publication: United States NLM ID: 7603509 Publication Model: Print Cited Medium: Internet ISSN: 1528-0020 (Electronic) Linking ISSN: 00064971 NLM ISO Abbreviation: Blood Subsets: MEDLINE
    • Publication Information:
      Publication: 2021- : [New York] : Elsevier
      Original Publication: New York, Grune & Stratton [etc.]
    • Subject Terms:
      Antigens, CD19/*immunology ; Hematopoietic Stem Cell Transplantation/*methods ; Immunologic Deficiency Syndromes/*therapy ; Receptors, Antigen, T-Cell, alpha-beta/*immunology; Adolescent ; Child ; Child, Preschool ; Graft Survival ; Graft vs Host Disease/immunology ; Graft vs Host Disease/prevention & control ; Histocompatibility Testing ; Humans ; Immunologic Deficiency Syndromes/immunology ; Infant ; Prospective Studies ; Treatment Outcome ; Unrelated Donors
    • Abstract:
      TCRαβ+/CD19+ graft depletion effectively prevents graft-versus-host disease (GVHD). In the current study, we compared the outcomes of hematopoietic stem cell transplantation (HSCT) with TCRαβ+/CD19+ depletion from matched unrelated donors (MUDs) and mismatched related donors (MMRDs) in patients with primary immunodeficiency (PID). A total of 98 pediatric patients with various PIDs underwent HSCT with TCRαβ+/CD19+ graft depletion from MUDs (n = 75) and MMRDs (n = 23). All patients received a fludarabine-/treosulfan-based conditioning regimen, with 73 also receiving a second alkylating agent. For GVHD prophylaxis, all but 2 received serotherapy (antithymocyte globulin) before HSCT and a short course of posttransplant immunosuppression. Neutrophil and platelet engraftment in both the MUD and MMRD groups occurred on days 14 and 13, respectively. The incidence of secondary graft failure was 0.16 and 0.17 (P = .85), respectively. The cumulative incidence of acute GVHD grade 2 to 4 was 0.17 in the MUD group and 0.22 in the MMRD group (P = .7). The incidence of cytomegalovirus (CMV) viremia was 0.5 in the MUD group and 0.6 in the MMRD group (P = .35). The frequency of CMV disease was high (17%), and the most common manifestation was retinitis. The kinetics of immune recovery was similar in both groups. The overall survival was 0.86 in the MUD group and 0.87 in the MMRD group (P = .95). In our experience, there was no difference in the outcomes of HSCT performed from MUD and MMRD. Hence, given the immediate availability of donors, in the absence of HLA-identical siblings, HSCT with TCRαβ+/CD19+ graft depletion from MMRDs can be considered as the first choice in patients with PID.
      (© 2019 by The American Society of Hematology.)
    • Comments:
      Comment in: Blood. 2019 Nov 14;134(20):1688-1689. (PMID: 31725867)
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    • Accession Number:
      0 (Antigens, CD19)
      0 (Receptors, Antigen, T-Cell, alpha-beta)
    • Publication Date:
      Date Created: 20190928 Date Completed: 20200302 Latest Revision: 20210202
    • Publication Date:
      20240829
    • Accession Number:
      PMC6856988
    • Accession Number:
      10.1182/blood.2019001757
    • Accession Number:
      31558465