title: 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.
  
  
  METHODS
  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.
authors.affiliation: Service of Hematology, Centre Hospitalier Metropole Savoie, 73011, Chambéry, France.
authors.forename: Marie
authors.initials: M
authors.lastname: Wrobel
authors.affiliation: Medical laboratory, Centre Hospitalier Metropole Savoie, 73011, Chambéry, France.
authors.forename: Emilie
authors.initials: E
authors.lastname: Comio
authors.affiliation: Haemophilia center and Transfusion medicine, Centre Hospitalier Metropole Savoie, 73011, Chambéry, France.
authors.forename: Valerie
authors.initials: V
authors.lastname: Gay
authors.affiliation: Service of Hematology, Centre Hospitalier Metropole Savoie, 73011, Chambéry, France.
authors.forename: Noureddine
authors.initials: N
authors.lastname: Baroudi
authors.affiliation: Service of Hematology, Centre Hospitalier Metropole Savoie, 73011, Chambéry, France.
authors.forename: Pascal
authors.initials: P
authors.lastname: Meyer
authors.affiliation: Hospital Pharmacy, Centre Hospitalier Metropole Savoie, 73011, Chambéry, France.
authors.forename: Christine
authors.initials: C
authors.lastname: Chuniaud-Louche
authors.affiliation: Service of Hematology, Centre Hospitalier Metropole Savoie, 73011, Chambéry, France.
authors.forename: Maya
authors.initials: M
authors.lastname: Hacini
authors.affiliation: Service of Hematology, Centre Hospitalier Metropole Savoie, 73011, Chambéry, France. gian-matteo.pica@ch-metropole-savoie.fr.
authors.forename: Gian Matteo
authors.initials: GM
authors.lastname: Pica
mesh_terms: D000368:Aged; D001779:Blood Coagulation Factors / Q000627:therapeutic use; D003029:Coagulants / Q000627:therapeutic use; D015942:Factor VIIa / Q000627:therapeutic use; D005938:Glucocorticoids / Q000627:therapeutic use; D006467:Hemophilia A / Q000097:blood / Q000150:complications* / Q000188:drug therapy; D006490:Hemostatics / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D015470:Leukemia, Myeloid, Acute / Q000150:complications*; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D010314:Partial Thromboplastin Time; D019106:Postoperative Hemorrhage / Q000188:drug therapy / Q000209:etiology*; D055728:Primary Myelofibrosis / Q000150:complications*; D000069283:Rituximab / Q000627:therapeutic use
chemical_list: D001779:Blood Coagulation Factors; D003029:Coagulants; D005938:Glucocorticoids; D006490:Hemostatics; D007166:Immunosuppressive Agents; C025667:prothrombin complex concentrates; D000069283:Rituximab; D015942:Factor VIIa; D008775:Methylprednisolone
keywords: Acquired hemophilia A; Activated prothrombin complex concentrates; Azacytidine; Myelofibrosis; Rituximab