Puerperal acquired factor VIII inhibitor causing a von Willebrand-like syndrome in a patient with anti-DNA antibodies.
A 30-year-old Malay lady, with no previous or family history of bleeding, presented with severe gum bleeding 25 days post-partum. The factor VIII:c was 0.03 iu/ml with evidence of a slow-acting factor VIII inhibitor. Von Willebrand factor antigen (VWF:age) varied from less that 0.05 to 0.17 iu/ml, and there was absent ristocetin-induced platelet aggregation. Anti-nuclear and anti-DNA antibodies were present, but there were no other features of systemic lupus erythematosus. There was some clinical response to cryoprecipitate and tranexamic acid, and slight improvement with corticosteroid. Fifteen months later, the patient has no active bleeding problem, and her VWF-ag is increasing spontaneously. However, factor VIII:c is less than 0.01 iu/ml and her factor VIII inhibitor titre is still > 20 Bethesda units/ml.
Jackson
N
N
Department of Medicine, Hospital Universiti Sains Malaysia, Kelantan.
Hashim
Z A
ZA
Zainal
N A
NA
Jamaluddin
N
N
D000328:Adult; D000974:Antibodies, Antinuclear / Q000097:blood*; D005169:Factor VIII / Q000037:antagonists & inhibitors* / Q000627:therapeutic use; D005260:Female; D005884:Gingival Hemorrhage / Q000209:etiology*; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications; D010974:Platelet Aggregation; D011239:Prednisolone / Q000627:therapeutic use; D011644:Puerperal Disorders / Q000209:etiology*; D014148:Tranexamic Acid / Q000627:therapeutic use; D014842:von Willebrand Diseases / Q000209:etiology*; D014841:von Willebrand Factor / Q000032:analysis
D000974:Antibodies, Antinuclear; D014841:von Willebrand Factor; D014148:Tranexamic Acid; D005169:Factor VIII; D011239:Prednisolone