抄録
Here reported is an anti-LAV/HTLV-III antibody positive hemophilia A case accompanied with idiopathic (immunological) thrombocytopenic purpura (ITP). The ITP of the case was succesfully treated with high-dose intravenous gammaglobulin.
The patient was a 42-y-o-man, having been recurrently treated with blood transfusions or factor VIII preparations, since diagnosed as hemophilia A (VIII: C 1%, APTT 99 sec.) in his infancy. In Nov. 1985, thrombocytopenia (36,000/cmm, and thereafter as low as 19,000) was pointed out. No possible causes of the thrombocytopenia, such as infection or drug administration could be identified. Although dysfunction of joints, subcutaneous bleeding and slight liver swelling were observed, the patient showed neither fever, lymphnode swelling, splenomegaly nor any skin diseases. As for the results of laboratory examinations, RBC, WBC, and megakaryocyte count in a bone marrow aspirate were all within normal limits. Nevertheless, HBs antibody, EB VCA IgG and LAV/HTLV-III antibody were all positive, and decrease of peripheral lymphocyte count as well as of OKT 4/8 ratio, and increase of serum γ-globulin and PAIgG levels were recognized. Autoantibodies were all negative. In March of the next year, high-dose intact γ-globulin was intravenously administered (400 mg/kg/day for successive 5 days). Consequently, the thrombocyte count immediately elevated up to 200,000/cmm, keeping the levels higher than 100,000/cmm even after one month. The peripheral lymphocyte count and OKT 4/8 ratio were slightly elevated, but no significant change could be found in the PA IgG level.
