Experience with factor VIII: C inhibitors and acquired von Willebrand's disease in an adult at Ramathibodi Hospital.
Eleven cases of acquired inhibitors against factor VIII: C and von Willebrand's factor (vWF) seen at the Department of Medicine, Ramathibodi Hospital from 1979 to 1991 were reviewed. Factor VIII: C inhibitor was found in 6 of 36 patients (17%) with hemophilia A (median age 18 years). Three patients each were weak (titer < 10 Bethesda units/ml), and strong antibody producers. Two cases of weak antibody producers had spontaneous disappearance of inhibitor, while all 3 strong antibody producers required specific treatment (corticosteroids, immunosuppressive drugs, and plasmapheresis). The inhibitor level temporarily declined in 2 patients, and disappeared in one. Spontaneous acquired inhibitor to factor VIII: C was seen in 3 patients. One each respectively had pemphigus vulgaris and bullous pemphigoid, autoimmune disease, and NIDDM. They were characterized by older age (median age 54 years), frequent skin and soft-tissue hematoma, but less hemarthroses. Inhibitor titer ranged from 15-280 Bethesda units/ml. Disappearance of the inhibitor after treatment with corticosteroids and immunosuppressive drugs were observed in all patients. Acquired von Willebrand's disease developed in 2 previously healthy patients. One patient was in the postpartum period, while the other had simultaneous acute viral hepatitis A infection. Both presented with the recent onset of spontaneous severe gingival bleeding, and demonstrated a prolonged bleeding time, reduced vWF:Ag (F VIIIR:Ag), and ristocetin cofactor (F VIIIR:vWF). Treatment with cryoprecipitate and corticosteroid resulted in remission of bleeding symptoms. Despite the rarity of these disorders, the recognition and proper management are of importance.
Angchaisuksiri
P
P
Division of Hematology, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Atichartakarn
V
V
Pathepchotiwong
K
K
Jootar
S
S
Ungkanont
A
A
Chuncharunee
S
S
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D005169:Factor VIII / Q000037:antagonists & inhibitors* / Q000276:immunology; D005260:Female; D006467:Hemophilia A / Q000150:complications* / Q000276:immunology / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007518:Isoantibodies / Q000097:blood*; D008297:Male; D008875:Middle Aged; D010956:Plasmapheresis; D013785:Thailand; D016896:Treatment Outcome; D014842:von Willebrand Diseases / Q000209:etiology* / Q000628:therapy
D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents; D007518:Isoantibodies; D005169:Factor VIII