Myelofibrosis and acquired hemophilia A: a case report

Abstract

Background: Myelofibrosis and acquired hemophilia A is a rare association. To the best of our knowledge only one case of myelofibrosis and acquired hemophilia A has been previously described.

Case presentation: A 66-year-old Caucasian man diagnosed with myelofibrosis evolving in acute myeloid leukemia was referred to us for postoperative bleeding. Hemostatic studies showed prolonged activated partial thromboplastin time, decreased factor VIII coagulation, and a high factor VIII inhibitor titer; these findings led to a diagnosis of acquired hemophilia A for which he was treated with methylprednisolone and recombinant activated factor VII on admission. Due to a lack of response he was subsequently treated with rituximab combined with activated prothrombin complex concentrates. Furthermore, he received azacytidine to treat the underlying hematological malignancies. Immunosuppressive rituximab therapy resolved acquired hemophilia A with marked efficacy.

Conclusions: Rapid and accurate diagnosis, effective hemostatic therapy, and timely treatment for underlying disease are important in the management of acquired hemophilia A secondary to hematological malignancy.

Keywords: Acquired hemophilia A; Activated prothrombin complex concentrates; Azacytidine; Myelofibrosis; Rituximab.

Fig. 1

This graph shows factor VIII inhibitor activity and factor VIII coagulant activity levels. The patient was treated with recombinant activated factor VII and activated prothrombin complex concentrate for 18 days. He was also given rituximab weekly for four administrations and one cycle of azacytidine, then factor VIII inhibitor disappeared and factor VIII coagulant activity gradually returned to normal. 5-AZA azacytidine, AAFVIII factor VIII inhibitor, aPCC activated prothrombin complex concentrate, VIII: C factor VIII coagulant activity