[Acquired hemophilia A accompanied by immune thrombocytopenia]

Kozue Yoshida, Ayako Yanagidani, Kazunori Murai, Hiroyuki Hamada, Akiyoshi Sato, Yasurou Miyairi, Masahiro Ieko, Yuji Wano
[Rinshō Ketsueki] the Japanese Journal of Clinical Hematology 2019, 60 (11): 1567-1572
A 72-year-old man was hospitalized because of thrombocytopenia (0.5×104 /µl) and anemia. The bone marrow test result revealed excessive numbers of megakaryocytes and no platelet adhesion. Furthermore, platelet-associated immunoglobulin G levels were high, and he was tested positive for Helicobacter pylori antibody. On the basis of these findings, immune thrombocytopenia was diagnosed. The patient was initially treated with eradication therapy; prednisolone, 20 mg/day (0.5 mg/kg) and a thrombopoietin receptor agonist 12.5 mg/day. During the course of treatment, the anemia worsened. Detailed examination revealed marked prolongation of activated partial thromboplastin time and inhibition of factor VIII activity. Therefore, he was diagnosed with acquired hemophilia A. Although extensive muscle hemorrhage had occurred, hemostatic therapy comprising intensification of the immunosuppressive therapy and administration of recombinant activated factor VII resulted in successful hemostasis. As the treatment progressed, inhibition of factor VIII recurred temporarily; however, immunosuppressive therapy was continued. No recurrence was observed even after 1 year of the onset of both diseases.

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