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

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-γ and TNF-α. 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.

journal_name

Blood

journal_title

Blood

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

doi

10.1182/blood-2011-04-351254

subject

Has Abstract

pub_date

2011-10-06 00:00:00

pages

3811-7

issue

14

eissn

0006-4971

issn

1528-0020

pii

blood-2011-04-351254

journal_volume

118

pub_type

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