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Logo of archdischArchives of Disease in ChildhoodVisit this articleSubmit a manuscriptReceive email alertsContact usBMJ
Arch Dis Child. May 1996; 74(5): 458–459.
PMCID: PMC1511550

CD40 ligand deficiency presenting as unresponsive neutropenia.


A male child presented with recurrent respiratory infections, otitis media, and oral ulceration and was found to be neutropenic. Investigations showed hypogammaglobulinaemia with normal serum IgM and a novel deletion in the gene for CD40 ligand on his X chromosome. Intravenous gammaglobulin did not lead to resolution of his neutropenia; G-CSF was also necessary.

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Selected References

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  • Korthäuer U, Graf D, Mages HW, Brière F, Padayachee M, Malcolm S, Ugazio AG, Notarangelo LD, Levinsky RJ, Kroczek RA. Defective expression of T-cell CD40 ligand causes X-linked immunodeficiency with hyper-IgM. Nature. 1993 Feb 11;361(6412):539–541. [PubMed]
  • Banatvala N, Davies J, Kanariou M, Strobel S, Levinsky R, Morgan G. Hypogammaglobulinaemia associated with normal or increased IgM (the hyper IgM syndrome): a case series review. Arch Dis Child. 1994 Aug;71(2):150–152. [PMC free article] [PubMed]
  • Banchereau J, Bazan F, Blanchard D, Brière F, Galizzi JP, van Kooten C, Liu YJ, Rousset F, Saeland S. The CD40 antigen and its ligand. Annu Rev Immunol. 1994;12:881–922. [PubMed]
  • Rieger CH, Moohr JW, Rothberg RM. Correction of neutropenia associated with dysgammaglobulinemia. Pediatrics. 1974 Oct;54(4):508–511. [PubMed]
  • Wang WC, Cordoba J, Infante AJ, Conley ME. Successful treatment of neutropenia in the hyper-immunoglobulin M syndrome with granulocyte colony-stimulating factor. Am J Pediatr Hematol Oncol. 1994 May;16(2):160–163. [PubMed]
  • Fasth A. Bone marrow transplantation for hyper-IgM syndrome. Immunodeficiency. 1993;4(1-4):323–323. [PubMed]

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