A case of factor VIII inhibitor-positive acquired hemophilia treated by plasmapheresis.
We report on a case of factor VIII inhibitor-positive acquired hemophilia in which combined therapy of plasma exchange (PE) and steroids was effective. The patient, a 68-year-old man, had undergone hemodialysis since April 1998, due to chronic renal failure caused by diabetic nephropathy. The hemostasis of blood access sites gradually became difficult after the initiation of dialysis and the prolongation of activated partial thromboplastin time (APTT) (74.5 s), and a decrease in factor VIII (0.02%) and an abnormally high concentration of factor VIII inhibitor (111 U/ml) were found. Under the diagnosis of factor VIII inhibitor-positive acquired hemophilia, 3 consecutive PE were performed, followed by a large dose administration of gamma globulin. However, the effect of this therapy disappeared within 20 days. Then the PE therapy was performed again accompanied by pulse methylprednisolone therapy. After that, factor VIII inhibitor was suppressed and the patient's hemostatic defect continued to improve even after the reduction of the steroid dose. These results suggest that PE is very effective in treating factor VIII inhibitor-positive acquired hemophilia.
Narukawa
N
N
Center of Blood Purification Therapy, Wakayama Medical College, Japan.
Abe
T
T
Shouno
M
M
Kitabata
Y
Y
Okamoto
M
M
Kobata
H
H
Akizawa
T
T
D000368:Aged; D003131:Combined Modality Therapy; D003928:Diabetic Nephropathies / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D005169:Factor VIII / Q000037:antagonists & inhibitors*; D005500:Follow-Up Studies; D006467:Hemophilia A / Q000209:etiology* / Q000628:therapy*; D006801:Humans; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000628:therapy; D008297:Male; D010531:Peritoneal Dialysis, Continuous Ambulatory / Q000009:adverse effects; D010956:Plasmapheresis / Q000379:methods*; D013256:Steroids / Q000627:therapeutic use*; D016896:Treatment Outcome
D013256:Steroids; D005169:Factor VIII