CLINICAL COURSE AND TREATMENT ON A HEMOPHILIA A PATIENT WITH FACTOR VIII INHIBITOR

• Published in: Journal of the Japan Society of Blood Transfusion Vol. 34; no. 3; pp. 319 - 325
• Main Authors: Deguchi, Akira, Suzuki, Hikozi, Wada, Hideo, Tsuda, Masayuki, Iwasaki, Eiichi, Deguchi, Katsumi, Mori, Yositaka, Shirakawa, Shigeru, Ohkubo, Itsuko
• Format: Journal Article
• Language: Japanese
• Published: The Japan Society of Transfusion Medicine and Cell Therapy 1988
• ISSN: 0546-1448; 1883-8383
• DOI: 10.3925/jjtc1958.34.319

A 40-year-old man had been suffered from bleeding tendency since childhood. At the age of 34, he was diagnosed as having hemophilia A when he was admitted for an operation of an uretheral stone. Two years after the diagnosis of hemophilia A, he was suspected of having factor VIII inhibitor, because the treatment of spontaneous bleeding in the right knee joint with factor VIII concentrate was unsuccessful. Partial thromboplastin time (PTT) and factor VIII activity before and after intravenous infusion of factor VIII concentrate were over 3min and 2.9% respectively. Further examination revealed a factor VIII inhibitor titer of 4.5 Bethesda unit. Eighteen months after detecting factor VIII inhibitor, he exhibited anamnestic reaction. Treatment with high doses of factor VIII concentrate were effective for his bleeding symptoms without improvement of PTT and factor VIII activity. Treatment with steroid, an immunosuppressive agent (azathioprine) and/or high-dose intact immunoglobulin, which were gave after the development of anamnestic reaction, did not decrease the factor VIII inhibitor titer. Therefore, it was guessed that he should be treated with prothrombin complex concentrate (PCC) and activated PCC.