Four cases are reported in which a bleeding disorder associated with a long bleeding time and a low factor-VIII Icvel, like von Willebrand's disease, appears to have developed in adult life. In each there were abnormalities of plasma proteins, similar to but in some instances more extreme than patterns found in hacmophilia and 'classical' von Willebrand's disease. One patient's bleeding tendency developed in association with a generalized illness, probably systemic lupus erythematosus, and improved rapidly on steroid treatment. The other three patients, with no symptoms of underlying disease, were not treated with steroids. Two patients, infused with plasma, showed rather more than the predicted rise in factor VIII. 'Classical' von Willebrand's discase is a familial, life-long bleeding disorder with a long blecding time and a low level of factor VIII. However, Simone et al(1968) described a similar but acquircd disorder arising in a child with systemic lupus erythematosus (SLE) in whom both conditions improved on steroid therapy; and Veltkamp et al(1g70) observed a like episode lasting 3 wceks in a man who drank a pesticide. This paper describes four unrelated cases in which a bleeding disorder with some laboratory fcatures of von Willebrand's disease developed spontaneously in adult life in patients who are known previously to have responded normally to major haelnostatic challenges.
MATERIALS AND METHODS
PaticntsThe four patients studied were: H.S., female, 60 yr, Mayo Clinic; E.Ca., male, 76 yr and H.G., male, 5 5 yr, St Thomas's Hospital, London; J.Co., male, 59 yr, Royal South Hams Hospital, Southampton. They were unrclated and none gave a family history of abnormal bleeding. Each proffered clear evidence of normal liaemostasis for at least the first four decades of life. The onset of the bleeding disorder became apparent from a change in the haeniostatic response to trauma, and E.Ca., H.G. and J.Co. also developed spontaneous blceding of the 'capillary' type, characterized by ecchymoses and bleeding from mucous membranes (Table I), for which no local cause was identified in any case.