Successful management of neutropenia in a patient with CD40 ligand deficiency by immunoglobulin replacement therapy

Iranian Journal of Allergy, Asthma and Immunology

Volumn 6, Issue 1, 2007, Pages 37-40

  Atarod, Lida ^a   Aghamohammadi, Asghar ^a,d   Moin, Mostafa ^a   Kanegane, Hiro ^b   Rezaei, Nima ^a   Kalantari, Kiara Rezaei ^a   Amirzagar, Ali Akbar ^c   Futatani, Takishi ^b   Miyawaki, Toshio ^b  

^a TEHRAN UNIVERSITY OF MEDICAL SCIENCES   (Iran)

^b TOYAMA MEDICAL AND PHARMACEUTICAL UNIVERSITY   (Japan)

^c TEHRAN UNIVERSITY OF MEDICAL SCIENCES   (Iran)

^d TEHRAN UNIVERSITY OF MEDICAL SCIENCES   (Iran)

Author keywords

Antibody deficiency; CD40 ligand; Hyper IgM syndrome; Intravenous immunoglobulin; Neutropenia

Indexed keywords

CD40 LIGAND; COTRIMOXAZOLE; GRANULOCYTE COLONY STIMULATING FACTOR; IMMUNOGLOBULIN;

ALKALINE PHOSPHATASE BLOOD LEVEL; ANAMNESIS; ANTIBIOTIC PROPHYLAXIS; ARTICLE; BONE MARROW BIOPSY; CASE REPORT; CHRONIC DISEASE; CLINICAL FEATURE; DISEASE SEVERITY; DNA EXTRACTION; DNA SEQUENCE; DRUG WITHDRAWAL; FOLLOW UP; GENE MUTATION; GENETIC POLYMORPHISM; HUMAN; HYPERIMMUNOGLOBULINEMIA M; INJECTION SITE SWELLING; LABORATORY TEST; MALE; MEDULLARY PAIN; NEUTROPENIA; PAIN; PRESCHOOL CHILD; RASH; SIDE EFFECT; SPLENOMEGALY; TREATMENT DURATION; TREATMENT OUTCOME; TREATMENT RESPONSE;

CD40 LIGAND; CHILD; GRANULOCYTE COLONY-STIMULATING FACTOR; HUMANS; IMMUNOGLOBULIN M; IMMUNOGLOBULINS, INTRAVENOUS; IMMUNOLOGIC DEFICIENCY SYNDROMES; MALE; NEUTROPENIA;

EID: 33847650123     PISSN: 17351502     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Article

References (18)

1. Rosen FS, Kevy SV, Merler E, Janeway CA, Gitlin D. Recurrent bacterial infections and dysgammaglobulinemia: deficiency of 7S gamma-globulins in the presence of elevated 19S gamma-globulins. Report of two cases. Pediatrics 1961; 28:182-95.
2. Notarangelo LD, Duse M, Ugazio AG. Immunodeficiency with hyper-IgM (HIM). Immunodefic Rev 1992; 3(2):101-21.
3. Notarangelo L, Casanova JL, Conley ME, Chapel H, Fischer A, Puck J. Primary immunodeficiency diseases: an update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee Meeting in Budapest, 2005. J Allergy Clin Immunol 2006; 117(4):883-96.
4. Uguz A, Yilmaz E, Ciftcioglu MA, Yegin O. An unusual presentation of immunodeficiency with hyper-IgM. Pediatr Dermatol 2001; 18(1):48-50.
5. Mori M, Nonoyama S, Neubauer M, Mitsuda T, Kosuge K, Yokota S. Mutation analysis and therapeutic response to granulocyte colony-stimulating factor in a case of hyperimmunoglobulin M syndrome with chronic neutropenia. J Pediatr Hematol Oncol 2000; 22(3):288-9.
6. Allen RC, Armitage RJ, Conley ME, Rosenblatt H, Jenkins NA, Copeland NG, et al. CD40 ligand gene defects responsible for X-linked hyper-IgM syndrome. Science 1993; 259(5097):990-3.
7. DiSanto JP, Bonnefoy JY, Gauchat JF, Fischer A, de Saint Basile G. CD40 ligand mutations in x-linked immunodeficiency with hyper-IgM. Nature 1993; 361(6412):541-3.
8. Korthauer U, Graf D, Mages HW, Briere F, Padayachee M, Malcolm S, et al. Defective expression of T-cell CD40 ligand causes X-linked immunodeficiency with hyper-IgM. Nature 1993; 361(6412):539-41.
9. Aruffo A, Farrington M, Hollenbaugh D, Li X, Milatovich A, Nonoyama S, et al. The CD40 ligand, gp39, is defective in activated T cells from patients with X-linked hyper-IgM syndrome. Cell 1993; 72(2):291-300.
10. Fuleihan R, Ramesh N, Loh R, Jabara H, Rosen RS, Chatila T, et al. Defective expression of the CD40 ligand in X chromosome-linked immunoglobulin deficiency with normal or elevated IgM. Proc Natl Acad Sci U S A 1993; 90(6):2170-3.
11. Wang WC, Cordoba J, Infante AJ, Conley ME. Successful treatment of neutropenia in the hyperimmunoglobulin M syndrome with granulocyte colonystimulating factor. Am J Pediatr Hematol Oncol 1994;1 6(2):160-3.
12. 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 ; 71(2):150-2.
13. Rezaei N, Farhoudi A, Pourpak Z, Aghamohammadi A, Moin M, Movahedi M, et al. Neutropenia in Iranian patients with primary immunodeficiency disorders. Haematologica 2005 ; 90(4):554-6.
14. Levy J, Espanol-Boren T, Thomas C, Fischer A, Tovo P, Bordigoni P, et al. Clinical spectrum of X-linked hyper-IgM syndrome. J Pediatr 1997; 131(1 Pt 1):47-54.
15. Ostenstad B, Giliani S, Mellbye OJ, Nilsen BR, Abrahamsen T. A boy with X-linked hyper-IgM syndrome and natural killer cell deficiency. Clin Exp Immunol 1997; 107(2):230-4.
16. Simon G, Marodi L. Successful treatment of cyclic neutropenia associated with hyperimmunoglobulin M syndrome using recombinant granulocyte-colony stimulating factor. Orv Hetil 1995; 136(40):2169-72.
17. Garcia-Perez MA, Paz-Artal E, Corell A, Moreno A, Lopez-Goyanes A, Garcia-Martin F, et al. Mutations of CD40 ligand in two patients with hyper-IgM syndrome. Immunobiology 2003; 207(4):285-94.
18. Durandy A, Honjo T. Human genetic defects in class-switch recombination (hyper-IgM syndromes). Curr Opin Immunol 2001; 13(5):543-8.