Myelofibrosis and acquired hemophilia A: a case report

Abstract

BackgroundMyelofibrosis 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 presentationA 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.ConclusionsRapid 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.

Use of Recombinant Factor VIIa for Bleeding Control in Treatment of Acute Extremity Compartment Syndrome Secondary to Primary Myelofibrosis

“…Probably, rFVIIa acts in a similar way in cases of postsurgical bleeding, enhancing the natural coagulation process to generate a "super-clot" formation that is more active and more noticeable thrombin burst at the injury site 12 . Acquired hemophilia A secondary to primary myelofibrosis has been reported 15 , but we could not detect such a coagulopathy in our patient. Therefore, with the intention to benefit from the super-clot formation, we administered high-dose rFVIIa for both controlling bleeding after the fasciotomy and preventing such a complication after the STSG procedure.…”

Acquired Hemophilia A: A Case Report

“…In their case, methylprednisolone was dosed at 1 mg/kg and rituximab was dosed at 375 mg/m 2 weekly for 4 weeks. 16 The methylprednisolone dose was a higher potency as compared to our prednisone dose; however, the rituximab dose was the same as ours.…”