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An Immunologic Evaluation of Hemophiliac Patients and Their Wives: Relationships to the Acquired Immunodeficiency Syndrome

RICHARD D. deSHAZO, M.D.; W. ABE ANDES, M.D.; JUDY NORDBERG, B.A.; JULIE NEWTON, B.S.; CAROLYN DAUL, M.D., Ph.D.; and BRIAN BOZELKA, Ph.D.
[+] Article and Author Information

Grant support: supported in part by a grant from the Louisiana Chapter of the National Hemophilia Foundation.

▸Requests for reprints should be addressed to W. Abe Andes, M.D.; Section of Hematology-Oncology, Tulane University School of Medicine; New Orleans, LA 70112.


New Orleans, Louisiana


© 1983 American College of PhysiciansAmerican College of Physicians


Ann Intern Med. 1983;99(2):159-164. doi:10.7326/0003-4819-99-2-159
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Recently, hemophiliac patients receiving factor VIII concentrate therapy have developed the acquired immunodeficiency syndrome. Because abnormalities of cell-mediated immunity are found in this syndrome, we evaluated the peripheral blood immunologic status of 24 patients with classic hemophilia and 5 patients with factor IX deficiency. Both groups had decreased percentages and numbers of helper-inducer lymphocytes (OKT4 + ) and increased percentages of suppressor-cytotoxic T- lymphocytes (OKT8 + ) which resulted in depressed OKT4/T8 ratios. Abnormalities of the T-lymphocyte subpopulation were most severe in 7 patients with factor VIII deficiency with lymphadenopathy who also had increased Ia+ cells and profoundly suppressed lymphocyte mitogenic responses. No correlation was found between OKT4/OKT8 ratios or lymphocyte responses to mitogen and the amount of factor VIII concentrate used per year. Evaluation of five wives of factor-VIII-deficient patients who had abnormal OKT4/ OKT8 ratios showed decreased percentages of OKT4 cells, but normal lymphocyte mitogenic responses. Serum levels of IgG were elevated in factor-VIII-deficient patients but not their wives or factor-IX-deficient patients. We conclude that T-lymphocyte subpopulation abnormalities and lymphocyte mitogenic responses are depressed in asymptomatic hemophiliac patients receiving either factor VIII or factor IX concentrates. These abnormalities are most severe in otherwise asymptomatic hemophiliac patients who have developed lymphadenopathy.

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