Abstract
A five-month-old boy presented with lower gastrointestinal bleed, recurrent infections and eczema. Blood picture revealed small platelets, high IgA, and IgM levels. A diagnosis of Wiskott-Aldrich Syndrome was made. The recent concepts in molecular pathology of the disease and treatment are discussed.
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References
Aldrich RA, Steinberg AG, Campbell DC. Pedigree demonstrating a sex-linked recessive condition characterized by draining ears, eczematoid dermatitis and bloody diarrhoea.Pediatrics 1954; 13: 133–139.
Inoue R, Kond N, Kuwabara N, Orii T. Aberrant patterns of immunoglobulin levels in Wiskott-Aldrich syndrome.Scand J Immunol 1955; 41: 188–193.
Murphy S, Ocki Fa, Naiman JL, Lusch CJ, Goldberg S, Gardner FH. Platelet size and kinetics in hereditary and acquired thrombocytopenia.N Engl J Med 1972; 286: 499–501.
Ten Bensel RW, Stadlan EM, Krivit W. The development of malignancy in the course of the Aldrich syndrome.J Pediatr 1966; 68: 761–767.
Perry GS, Spector BD, Schuman LM, Mandel JS, Anderson VE, McHugh RBel al. The Wiskott-Aldrich syndrome in the United States and Canada (1892–1979).J Pediatr 1980; 97: 72–78.
Sullivan KE, Mullen CA, Blaese RM, Winkelstein JA. A multi-institutional survey of the Wiskott-Aldrich syndrome.J Pediatr 1994; 125: 876–885.
Siminovitch KA, Greer WL, Novogrodsky A, Axelsson B, Somani AK, Peacocke M. A diagnostic Assay for the Wiskbtt-Aldrich syndrome and its variant forms.J Investig Med 1995; 43: 159–169.
Jonathan M, Derry J, Ochs HD, Francke U. Isolation of novel gene mutated in Wiskott-Aldrich syndrome.Cell 1994; 78: 635–644.
Parkman R, Remold O’Donnel E, Kenney DM, Perrine S, Rosen FS. Surface protein abnormalities in lymphocytes and platelets from patients with Wiskott-Aldrich syndrome.Lancet 1981; 2: 1387–1389.
Kolluri R, Shehabeldin A, Peacocke M, Lambronwah AM, Weissman MS, Siminovitch KAet al. Identification of Wasp mutations in patients with Wiskott-Aldrich syndrome and isolated thrombocytopenia reveals allelic heterogeneity at the WAS locus.Hum Mol Genet 1995; 4: 1119–1126.
Lum LG, Tubergen DG, Corash L, Blease RM. Splenectomy in the management of the thrombocytopenia of the Wiskott-Aldrich syndrome.N Engl J Med 1980; 302: 892–896.
Parkman R, Rappaport J, Geha R, Belli J, Cassady R, Levey Ret al. Complete correction of the Wiskott-Aldrich syndrome by allogenic bone marrow transplantation.N Engl J Med 1978; 298: 921–927.
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Srivastava, A., Swaid, H.A., Kabra, M. et al. Management of Wiskott-Aldrich syndrome. Indian J Pediatr 63, 709–712 (1996). https://doi.org/10.1007/BF02730830
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DOI: https://doi.org/10.1007/BF02730830