Effectiveness of plasma exchange for acquired type A hemophilia

CEN Case Reports
Sawako GotoKaoru Tabei

Abstract

An 80-year-old man was treated at our hospital for chronic kidney disease, diabetes mellitus and hypertension. He presented a bleeding tendency over the past several weeks. When he was admitted to our hospital with a consciousness disturbance, he had irregular 10-20 cm patches of subcutaneous bleeding. Laboratory findings showed prolonged activated partial thromboplastin time (APTT) but normal platelet count and prothrombin time. The administration of fresh frozen plasma did not control the bleeding tendency and the result of APTT cross-mixing test suggested the presence of factor VIII inhibitor. The diagnosis of acquired hemophilia A was made by a marked decrease in factor VIII activity levels (<1 %) and a high titer of factor VIII inhibitor (11 Bethesda units). To remove the factor VIII inhibitor and suppress its production, plasma exchange was performed and methylprednisolone was administered. Thereafter, the bleeding tendency could be controlled with APTT measurement normalization. Plasma exchange does not have a first priority in the treatment but our case findings suggested that plasma exchange is an effective method for eliminating factor VIII inhibitor and alleviating acquired hemophilia A severity.

References

Dec 23, 1999·Therapeutic Apheresis : Official Journal of the International Society for Apheresis and the Japanese Society for Apheresis·H OgataT Akizawa
Apr 3, 2003·British Journal of Haematology·Julio DelgadoAna Villar
Oct 10, 2009·British Journal of Haematology·Peter W Collins, Charles L Percy
Feb 7, 2014·Therapeutic Apheresis and Dialysis : Official Peer-reviewed Journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy·Heike ZeitlerGeorg Goldmann

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