Impaired immune function in hemophilia patients treated exclusively with cryoprecipitate: relation to duration of treatment.
Recently, abnormalities of cell-mediated immunity were found in hemophiliac patients receiving factor VIII concentrate therapy. Contradictory results were reported concerning cellular immune functions in hemophiliacs treated only with cryoprecipitate or fresh frozen plasma. Therefore, we evaluated the immunological status of 15 Israeli patients with severe classic hemophilia-A who were treated only with cryoprecipitate and never exposed to factor VIII concentrate whether of commercial source or blood bank prepared. As a group, only mildly depressed cellular immune functions and slight reduction in the helper to suppressor cell ratio were found. However, when patients treated more than 15 years were analyzed separately, a significant reduction in proportion of T cells, T-helper cells, helper to suppressor ratio, and proliferative response to phytohemaglutinin and pokeweed mitogen were observed compared to patients treated for less than 15 years and normal controls. Proportion of T-suppressor cells, Con A-activated suppressor activity, and IgG and IgA levels were significantly elevated in patients treated for more than 15 years. These results may support the view that derangement of immune function in hemophiliacs results from infusion of foreign proteins or an ubiquitous virus rather than contracting AIDS infectious agent.
D000163:Acquired Immunodeficiency Syndrome / Q000209:etiology; D000293:Adolescent; D000328:Adult; D002648:Child; D005169:Factor VIII / Q000627:therapeutic use*; D005340:Fibrinogen / Q000627:therapeutic use*; D006467:Hemophilia A / Q000276:immunology* / Q000628:therapy; D006801:Humans; D007111:Immunity, Cellular; D008214:Lymphocytes / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D013997:Time Factors; D065227:Transfusion Reaction
C026912:cryoprecipitate coagulum; D005169:Factor VIII; D005340:Fibrinogen