Acquired hemophilia A in a patient with essential thrombocythemia.
A 69-year-old woman with essential thrombocythemia (ET) developed giant ecchymosis, and she was admitted to hospital. Marked anemia (Hb 8.1 g/dl) accompanied by a prolonged activated partial thromboplastin time (89.6 s) was observed, and she received red blood cells (RBC) and fresh frozen plasma (FFP). On day 2 after admission, consciousness disturbance suddenly occurred, whereas computed tomography of the brain showed no evidence of bleeding. As the ecchymosis progressed, she developed shock. Although RBC and FFP transfusions were administered, she developed multi-organ failure and died 48 h after admission. Low factor VIII activity (<1%) accompanied by factor VIII inhibitor (17 Bethesda units) was found after her death. An autopsy revealed cerebral infarction without cerebral herniation. To date, acquired hemophilia A accompanying ET has been described in only one other patient. Although acquired factor VIII inhibitor is a rare disease, it should be tested for in ET patients with marked hemorrhagic tendency.
Department of Hematology, Tokyo Women's Medical University, School of Medicine, Japan. mori@dh.twmu.ac.jp
Naoki
N
Mori
Kyoichi
K
Totsuka
Noriko
N
Ishimori
Kentaro
K
Yoshinaga
Masanao
M
Teramura
Sanshiro
S
Noguchi
Hideaki
H
Oda
Toshiko
T
Motoji
D000368:Aged; D002544:Cerebral Infarction / Q000473:pathology; D004438:Ecchymosis / Q000209:etiology; D005169:Factor VIII / Q000037:antagonists & inhibitors; D005260:Female; D006467:Hemophilia A / Q000150:complications* / Q000175:diagnosis*; D006801:Humans; D013920:Thrombocythemia, Essential / Q000150:complications* / Q000175:diagnosis*; D014057:Tomography, X-Ray Computed
D005169:Factor VIII