Very Rare Case of Acquired Hemophilia A and Associated Myelofibrosis Reported in Patient

Malika Ammam, PhD avatar

by Malika Ammam, PhD |

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Researchers at the Centre Hospitalier Metropole Savoie in France identified a rare case of associated myelofibrosis (MF) and acquired hemophilia A (AHA), which they believe to be only the second case reported to date. The study, “Myelofibrosis and acquired hemophilia A: a case report,” was published in the Journal of Medical Case Reports.

MF and AHA are both blood disorders, with MF characterized by a disruption in the normal production process of blood cells in bone marrow, and AHA by autoantibodies forming against a clotting protein — factor VIII (FVIII) — to cause bleeding problems. Both conditions may result in severe anemia, fatigue, and weakness.

Association of both conditions in the same patient is quite rare, making it difficult to establishing a proper diagnosis. “MF associated with AHA is a challenge to diagnose, considering that AHA is still not a widely known condition,” the authors wrote.

In this case report, a 66-year-old Caucasian man was admitted to the hospital for postoperative bleeding. He was diagnosed with MF developing in acute myeloid leukemia, a blood cancer characterized by the accelerated growth of abnormal white blood cells, which build up in bone marrow and interfere with the formation of normal blood cells.

Hemostatic treatments (used to stop bleeding) revealed a prolonged activated partial blood coagulation time, reduced factor VIII coagulation, and elevated levels of factor VIII inhibitor. These findings helped in diagnosing the patient with AHA. A treatment based on methylprednisolone and recombinant activated factor VII was then administrated to the man, but he responded poorly. A second attempt using a combination treatment, based on  rituximab and activated prothrombin complex concentrates, was given. Because the patient was also suffering  from hematological malignancies, he was put on azacytidine therapy as well.

The rituximab-based therapy was efficient in treating AHA in the patient. “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,” the authors concluded.