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Partial immune reconstitution of X-linked hyper IgM syndrome with recombinant CD40 ligand

  1. Author:
    Jain, A.
    Kovacs, J. A.
    Nelson, D. L.
    Migueles, S. A.
    Pittaluga, S.
    Fanslow, W.
    Fan, X. Y.
    Wong, D. W.
    Massey, J.
    Hornung, R.
    Brown, M. R.
    Spinner, J. J.
    Liu, S. Y.
    Davey, V.
    Hill, H. A.
    Ochs, H.
    Fleisher, T. A.

  2. Author Address

    [Jain, Ashish; Fan, Xiying; Massey, Justin; Spinner, Jacob J.; Liu, Shuying] NIAID, Host Def Lab, NIH, Bethesda, MD 20892 USA. [Kovacs, Joseph A.] NIH, Dept Crit Care Med, Ctr Clin, Bethesda, MD 20892 USA. [Nelson, David L.] NCI, Metab Branch, Bethesda, MD 20892 USA. [Migueles, Stephen A.] NIAID, Immunoregulat Lab, NIH, Bethesda, MD 20892 USA. [Pittaluga, Stefania] NCI, Pathol Lab, Bethesda, MD 20892 USA. [Fanslow, William] Amgen Inc, Seattle, WA USA. [Wong, Duane W.] Arizona Allergy Associates, Phoenix, AZ USA. [Hornung, Ronald] NCI Frederick, Clin Serv Program, SAIC Frederick Inc, NIH, Bethesda, MD USA. [Brown, Margaret R.; Fleisher, Thomas A.] NIH, Dept Lab Med, Ctr Clin, Bethesda, MD 20892 USA. [Davey, Victoria] NIAID, Off Clin Director, NIH, Bethesda, MD 20892 USA. [Hill, Harry A.] Univ Utah, Dept Pediat, Salt Lake City, UT USA. [Ochs, Hans] Univ Washington, Dept Pediat, Seattle, WA 98195 USA.;Jain, A (reprint author), NIAID, Host Def Lab, NIH, 10 Ctr Dr,Rm 5W 3950, Bethesda, MD 20892 USA;ajain@nih.gov
    1. Year: 2011
    2. Date: Oct
  2. Journal: Blood
    1. 118
    2. 14
    3. Pages: 3811-3817
  4. Type of Article: Article
  5. ISSN: 0006-4971
  1. Abstract:

    X-linked hyper IgM syndrome (XHM) is a combined immune deficiency disorder caused by genetic alterations in CD40 ligand. The purpose of this study was to investigate the safety and efficacy of recombinant CD40 ligand (rCD40L) in the treatment of the disease. Three children were administered rCD40L subcutaneously 3 times per week at 0.03 mg/kg for 22 weeks, and after a 12-week drug-free interval, the dose was increased to 0.05 mg/kg for an additional 22 weeks of treatment. Although specific antibody responses to T cell-dependent antigens was lacking, administration of rCD40 resulted in acquisition of the capacity to mount cutaneous delayed type hypersensitivity reactions that disappeared during the drug-free interval as well as the postbiologic follow-up period. With rCD40L treatment, patient T cells developed a new capacity to respond to T-cell mitogens with synthesis of IFN-gamma and TNF-alpha. Intracellular cytokine staining studies showed that both CD4(+) and CD8(+) T cells participated in this response. Finally, CD40L therapy was associated with changes in lymph node size and architecture based on comparison of biopsies taken before and after therapy. This clinical study showed that rCD40L is capable of improving T cell-immune function in patients with XHM. (Blood. 2011; 118(14):3811-3817)

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External Sources

  1. View Full Text
  2. DOI: 10.1182/blood-2011-04-351254
  3. View record in Web of Science®
  4. WOS: 000295807700012

Library Notes

  1. Fiscal Year: FY2011-2012
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