咽頭出血を初発とした後天性血友病例

概要

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Hemophilia is conventionally monogenic causing hemorrhagic symptoms due to decreased or deficient factor VIII. Acquired hemophilia (HA), however develops clinical hemophilic analogous symptom when auto antibody factor VIII or IX develops. This rare disorder, which causes pharyngeal hemorrhage has not been reported in Japan insofar as we know until our case. An 83-year-old Japanese woman seen for acute pharyngalgia and hematemesis lasting two months. Required emergency tracheotomy for hematoma airway narrowing, but bleeding persisted, after an operation. We finally diagnosed her condition as acquired hemophilia because of aberrant bleeding, prolonged coagulation time (APTT), and decreased factor VIII and inhibitor-positive factor VIII. For inhibitor removal and hemostasis, we administered prednisolone (PSL), factor VIII concentrates, and genetic recombination activated factor VII (rFVVII). Factor VIII inhibitor showed loss than start of therapy in two months and follow-up has proceeded hemorrhage-free as of this writing.
2011-06-01