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

Authors: Jain, A  Kovacs, JA  Nelson, DL  Migueles, SA  Pittaluga, S  Fanslow, W  Fan, X  Wong, DW  Massey, J  Hornung, R  Brown, MR  Spinner, JJ  Liu, S  Davey, V  Hill, HA  Ochs, H  Fleisher, TA 
Citation: Jain A, etal., Blood. 2011 Aug 12.
Pubmed: (View Article at PubMed) PMID:21841160
DOI: Full-text: DOI:10.1182/blood-2011-04-351254

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 three 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 dependant 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 post-biologic 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.

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RGD Object Information
RGD ID: 5490298
Created: 2011-09-13
Species: All species
Last Modified: 2011-09-13
Status: ACTIVE



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RGD is funded by grant HL64541 from the National Heart, Lung, and Blood Institute on behalf of the NIH.