[Acquired factor VIII hemophilia in a geriatric patient].
Acquired hemophilia is a rare coagulopathy in adults, associated with bleeding complications. Although the etiology of this disorder remains obscure, an autoimmune mechanism produces the development of autoantibodies against factor VIII. About half of cases are associated with other conditions, mainly post-partum, underlying cancer, autoimmune disease. An 81-year-old male was admitted to the hospital with extensive hematomas (neck, chest, arms and lower limbs). There was no family or personal history of congenital bleeding diathesis. He had chronic bronchitis and cerebrovascular disease; no drugs had been used during the month prior to noted symptoms. Laboratory parameters revealed: hemoglobin 10.9 g%, normal platelet count and white blood cells, prolonged activated partial thromboplastin time (98 s), with normal prothrombin time and fibrinogen concentration. An activated partial thromboplastin time mixing study did not show any correction, suggesting a coagulation inhibitor. Lupus anticoagulant and anticardiolipin antibodies were negative. Biochemical, immunological tests and tumor markers were normal. Thoracic and abdominal computed tomographic scan did not reveal pathological images or hematomas. Analysis of clotting factors revealed decreased factor VIII (< 2%) and elevated factor VIII inhibitor (55 Bethesda units). Idiopathic acquired hemophilia diagnosis was made. Red blood cell transfusion and human factor VIII (2000 U/day for 7 days) infusion were initiated, intravenously with methylprednisolone. A progressive improvement in clinical conditions and laboratory parameters was observed. After 18 days the patient was discharged and treated with prednisone. At follow-up control the clinical conditions and laboratory parameters were normal.
Boddi
Walter
W
U.O. di Medicina Interna, Ospedale Campostaggia, ASL 7 di Poggibonsi. w.boddi@usl7.toscana.it
Machetti
Stefania
S
Sodi
Nicola
N
Sammicheli
Lucia
L
Cati
Graziella
G
Marotta
Giovanna
G
Sabatini
Laura
L
Cappelli
Roberto
R
D000369:Aged, 80 and over; D001323:Autoantibodies / Q000097:blood; D017707:Erythrocyte Transfusion / Q000379:methods; D005169:Factor VIII / Q000037:antagonists & inhibitors*; D005938:Glucocorticoids / Q000627:therapeutic use; D006467:Hemophilia A / Q000188:drug therapy / Q000276:immunology* / Q000628:therapy*; D006801:Humans; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D016896:Treatment Outcome
D001323:Autoantibodies; D005938:Glucocorticoids; D005169:Factor VIII; D008775:Methylprednisolone