A severe haemorrhagic disorder with prolonged bleeding time due to a plasma defect but with normal factor VIII.

Abstract

Abstract A young girl had bleeding symptoms resembling those of severe von Willebrand's disease. At 12 years of age she had perilous menorrhagia. The Duke bleeding time usually exceeded 30 min, but all coagulation factors were normal and, unlike what is seen in von Willebrand's disease, factor VIII was normal. There was no pathologic fibrinolysis. The platelet adhesiveness was near the low limit of the normal range when tested with Hellem's whole blood method, and normal when examined with his plasma‐ADP method, but decreased when assessed with Salzman's method. The platelets appeared normal and aggregated and adhered normally to a glass slide. Aggregation after addition of ADP or connective tissue suspension was also normal. The prothrombin consumption test was usually normal. Fraction I‐0, fresh plasma, and stored plasma shortened the bleeding time. There was no family history of a bleeding disorder, and her parents showed no evidence of such a condition.The findings suggest a special bleeding disorder of a new type resembling severe von Willebrand's disease but distinctly different from it. We have provisionally called the condition morbus Rita.