Acquired von Willebrand's disease in myelofibrosis and essential thrombocythemia

Abstract

J. GOYAL,* V. V. B. REDDY† and M. B. MARQUES†*Department of Medicine, University of Alabama at Birmingham, Birmingham, AL; and †Department of Pathology, University of Alabama atBirmingham, Birmingham, AL, USAAcquired von Willebrand’s disease (AVWD) is a rare coagulopathysecondary to a variety of conditions such as hypothyroidism, abnormalheart valves and lymphoproliferative and autoimmune disordersamong others [1]. AVWD, like the inherited form of VWD, may becharacterized by quantitative or qualitative abnormalities of vonWillebrand factor (VWF). Few reports have linked AVWD to myelo-proliferative neoplasms (MPN) [2]. We hereby report a patient withAVWD in association with essential thrombocythemia (ET) andmyelofibrosis, who was asymptomatic. In such cases, unless otherwisesuspected and tested for, the condition may be missed and bleedingcould ensue if an invasive procedure becomes necessary. We haveattempted to highlight the importance of keeping AVWD in mindwhenever significant thrombocytosis is present. Although bleeding/thrombotic complications do not occur in all patients with this presen-tation, early recognition and prompt treatment are important [3].