Abstract
Sequential studies of cellular and humoral immunity were conducted in an infant with the Wiskott-Aldrich syndrome prior to and after a splenectomy for uncontrollable hemorrhage. All measures of cellular immunity showed gradual improvement during the 8-month period after surgery. Serum isohemagglutinins, diphtheria and tetanus antibodies, and the percentage of immunoglobulin-bearing B cells did not change significantly from presplenectomy values. The serum IgE concentration declined from a high of 10,800 IU/ml at 1 month post-splenectomy to a low of 860 IU/ml at 5 months after surgery and the IgG concentration gradually decreased from a high of 1880 mg/dl presplenectomy to a low of 620 mg/dl 8 months later. The platelet count ranged from 64,000 to 206,000/mm3 for the first 6 months after splenectomy. It decreased precipitously 6.5 months after the operation; at the same time there was a marked rise in platelet-bound IgG antibody (PB-IgG). The PB-IgG declined rapidly following vincristine therapy and, after another rise, declined more gradually following steroid therapy.
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Aldrich RA, Steinberg A, Campbell DC: Pedigree demonstrating a sex-linked recessive condition characterized by draining ears, eczematoid dermatitis and bloody diarrhea. Pediatrics 13:133–138, 1954
Huntley CC, Dees SC: Eczema associated with thrombocytopenic purpura and purulent otitis media. Pediatrics 19:351–360, 1957
Wolff JA: Wiskott-Aldrich Syndrome: Clinical, immunological and pathologic observations. J Pediatr 70:221–232, 1967
Cooper MD, Chase HP, Lawman JT, Krivit W, Good RA: Wiskott-Aldrich Syndrome: An immunologic deficiency disease involving the afferent limb of immunity. Am J Med 44:499–511, 1968
Blaese RM, Strober W, Brown RS, Waldmann TA: The Wiskott-Aldrich Syndrome: A disorder with a possible defect in antigen processing or recognition. Lancet 1:1056–1061, 1968
Blaese RM, Strober W, Waldmann TA: Immunodeficiency in the Wiskott-Aldrich Syndrome. Birth Defects 11:250–254, 1975
Oppenheim JJ, Blaese RM, Waldmann TA: Defective lymphocyte transformation and delayed hypersensitivity in Wiskott-Aldrich Syndrome. J Immunol 104:835–844, 1970
Spitler LE, Levin AS, Stites DP, Fudenberg HH, Huber H: The Wiskott-Aldrich Syndrome: Immunologic studies in nine patients and selected family members. Cell Immunol 19:201–218, 1975
Blaese RM, Strober W, Levy AL, Waldmann TA: Hypercatabolism of IgG, IgA, IgM and albumin in the Wiskott-Aldrich Syndrome. J Clin Invest 50:2331–2338, 1971
Radl J, Dooren LJ, Morell A, Skvaril F, Vossen MJJ, Vittenbogaart CH: Immunoglobulins and transient paraproteins in sera of patients with the Wiskott-Aldrich Syndrome: A follow-up study. Clin Exp Immunol 25:256–263, 1976
Filipovich AH, Krivit W, Kersey JH, Burke BA: Fatal arthritis as a complication of Wiskott-Aldrich Syndrome. J Pediatr 95:742–744, 1979
Kersey JH, Spector BD, Good RA: Primary immunodeficiency diseases and cancer: The immunodeficency-cancer registry. Int J Cancer 12:333–347, 1973
Baldini MG: Nature of the platelet defect in the Wiskott-Aldrich Syndrome. Ann NY Acad Sci 201:437–444, 1972
Ochs HD, Slichter SJ, Harker LA, von Behrens WE, Clark RA, Wedgwood RJ: The Wiskott-Aldrich Syndrome: Studies of lymphocytes, granulocytes and platelets. Blood 55:243–252, 1980
Lum LG, Tubergen DG, Carcish L, Blaese RM: Splenectomy in the management of the thrombocytopenia of the Wiskott-Aldrich Syndrome. N Engl J Med 302:892–896, 1980
Weiden PL, Blaese RM: Hereditary thrombocytopenia: Relation to Wiskott-Aldrich Syndrome with special reference to splenectomy. J Pediatr 80:226–234, 1972
Buckley RH, Dees SC, O'Fallon WM: Serum immunoglobulins. I. Levels in normal children and in uncomplicated childhood allergy. Pediatrics 41:600–601, 1968
Buckley RH, Fiscus SA: Serum IgD and IgE concentrations in immunodeficiency diseases. J Clin Invest 55:157–165, 1975
Wegman TG, Smithies O: A simple hemagglutination system requiring small amounts of red cells and antibodies. Transfusion 6:67–73, 1966
Buckley RH, Dees SC: Serum immunoglobulins. III. Abnormalities associated with chronic urticaria in children. J Allergy 40:294–303, 1967
Schiff RI, Buckley RH, Gilbertsen RB, Metzgar RS: Membrane receptors and in vitro responsiveness of lymphocytes in human immunodeficiency. J Immunol 112:376–386, 1974
Kaplow LS: Simplified myeloperoxidase stain using benzidine dihydrochloride. Blood 26:215–219, 1965
Dixon R, Rosse W, Ebbert L: Quantitative determination of antibody in idiopathic thrombocytopenic purpura: Correlation of serum and platelet-bound antibody with clinical response. N Engl J Med 292:230–236, 1975
Kaden BR, Rosse WF, Hauch TW: Immune thrombocytopenia in lymphoproliferative diseases. Blood 53:545–551, 1979
McGeady SJ, Buckley RH: Depression of cell-mediated immunity in atopic eczema. J Allergy Clin Immunol 56:393–406, 1975
Tada T, Okumura K: The role of antigen-specific T cell factors in the immune response. Adv Immunol 28:1–87, 1979
Parkman R, Rappaport J, Geha R, Bello J, Cassady R, Levey R, Nathan DG, Rosen FS: Complete correction of the Wiskott-Aldrich Syndrome by allogeneic bone marrow transplantation. N Engl J Med 298:921–927, 1978
Ahn YS, Harrington WJ, Seelman RC, Eytel CS: Vincristine therapy of idiopathic and secondary thrombocytopenias. N Engl J Med 291:376–380, 1974
Ahn YS, Harrington WJ: Treatment of idiopathic thrombocytopenic purpura (ITP). Ann Rev Med 28:299–309, 1977
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Knutsen, A.P., Rosse, W.F., Kinney, T.R. et al. Immunologic studies before and after splenectomy in a patient with the Wiskott-Aldrich syndrome. J Clin Immunol 1, 13–19 (1981). https://doi.org/10.1007/BF00915472
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DOI: https://doi.org/10.1007/BF00915472