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Leukocyte Transfusion-Associated Granulocyte Responses in a Patient with X-Linked Hyper-IgM Syndrome

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Abstract

X-linked hyper-IgM syndrome (XHIM) is a severe congenital immunodeficiency caused by mutations in CD154 (CD40 ligand, gp39), the T cell ligand for CD40 on B cells. Chronic or cyclic neutropenia is a frequent complicating feature that heightens susceptibility to severe infections. We describe a patient with a variant of XHIM who produced elevated levels of serum IgA as well as IgM and suffered from chronic severe neutropenia. Eight of ten leukocyte transfusions with cells from a maternal aunt, performed because of mucosal infections, resulted in similar episodes of endogenous granulocyte production. Transfection studies with the mutant CD154 protein indicate that the protein is expressed at the cell surface and forms an aberrant trimer that does not interact with CD40. The data suggest that allogeneic cells from the patient's aunt, probably activated T cells bearing functional CD154, may interact with CD40+ recipient cells to produce maturation of myeloid precursors in the bone marrow.

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Authors and Affiliations

  1. Department of Pediatrics, University of Alabama at Birmingham, Birmingham, Alabama

    T. Prescott Atkinson, Carol A. Smith, Thomas H. Howard & Max D. Cooper

  2. Biogen, Inc., 12 Cambridge Center, Cambridge, Massachusetts

    Yen-Ming Hsu, Ellen Garber & Lihe Su

  3. Department of Medicine, University of Alabama at Birmingham, Birmingham, Alabama

    Josef T. Prchal, Michael P. Everson & Max D. Cooper

  4. Department of Pathology, University of Alabama at Birmingham, Birmingham, Alabama

    Max D. Cooper

  5. Department of Microbiology, University of Alabama at Birmingham, Birmingham, Alabama

    Max D. Cooper

Authors
  1. T. Prescott Atkinson
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  2. Carol A. Smith
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  3. Yen-Ming Hsu
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  4. Ellen Garber
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  5. Lihe Su
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  6. Thomas H. Howard
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  7. Josef T. Prchal
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  8. Michael P. Everson
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  9. Max D. Cooper
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Atkinson, T.P., Smith, C.A., Hsu, YM. et al. Leukocyte Transfusion-Associated Granulocyte Responses in a Patient with X-Linked Hyper-IgM Syndrome. J Clin Immunol 18, 430–439 (1998). https://doi.org/10.1023/A:1023286807853

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  • DOI: https://doi.org/10.1023/A:1023286807853

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