Spontaneous recurrent hematuria and hematospermia: Unique manifestations of von Willebrand disease type I. Case report

Abstract

In this report we describe the case of a patient with unrecognized von Willebrand disease (vWD), in whom the only presenting symptoms were spontaneous and recurrent hematuria with bladder tamponade, associated with recurrent hematospermia. The diagnosis was made only after several admissions to the hospital. We suggest to include coagulopathies such as vWD as part of the evaluation in patients with unexplained genito-urinary bleeding.KEY WORDS: von Willebrand disease; Hematuria; Hematospermia. SummaryNo conflict of interest declared., the most common form, accounting for up to 80% of reported cases, and is generally transmitted as an autosomal dominant disorder. Mucocutaneous bleeding is the most common symptom in these patients, and typically presents as epistaxis, easy bruising, menorrhagia, gingival bleeding and post-traumatic or postsurgical bleeding (4). To date very few cases of spontaneous hematuria and hematospermia have been reported (5, 6); they were secondary to trauma such as self-instrumentation, catheterization, falls or straddle injuries. In this report we describe a patient with unrecognized vWD, in whom the only presenting symptoms were spontaneous and persistent hematuria and hematospermia. CASE REPORTA 34 years old male patient, with chronic adrenal failure under substitutive therapy, came to our observation because of spontaneous and persistent hematuria and hematospermia, recurring since a few years; the hematuria was so important that in few occasions he underwent to acute urinary retention and bladder tamponade. He was already been evaluated in other Urologic Units, where investigations were performed; he had renal, bladder, and prostatic ultrasound, abdominal CT scan and cystoscopy, that showed normal morphology and function of the urinary tract; urine and semen colture did not show any infection. In the family history, no hemorrhagic disease was present. He was admitted from Emergency to our Department of Urology for further investigations and treatment, after another episode of hematuria with bladder tamponade and acute urinary retention. We performed routine blood exams, that were normal, and a urethrocystoscopy, that did not show any abnormality; renal and bladder ultrasound was normal, while prostatic ultrasound showed only a slight periurethral congestion and oedema, with symmetric seminal vesicles. An hematologic consultation was then requested; coagulation parameters PT, PTT, Fibrinogen and Platelet count were normal, as well as the platelet agglutination test with Ristocetin; in the suspicion of an hematologic disorder further investigations were performed; it was pos-

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