Management of Severe Gastrointestinal Bleeding in a Patient with Acquired Von Willebrand Disease Complicating Enteropathic-Associated T-Cell Lymphoma

摘要

Acquired von Willebrand's disease (v WD) is a rare bleeding disorder with clinical and laboratory features mimicking the congenital form of the disease. An abnormal bleeding time, decreased levels of both factor VIII and von Willebrand factor (v WF), and moderate to severe mucosal bleeding characterize both forms of the disease. Acquired v WD occurs sporadically with no family history of v WD and usually with no previous coagulopathy. It is oftenlinked with immune system dysfunctions and particularly with clonal hematoproliferative diseases, including Enteropathic-Associated T-cell Lymphomas (EATL). Treatment of acquired v WD is often difficult and requires either correcting the acute bleeding episode or treating the associated condition. This case report supports the use of cyclophosphamide, doxorubicin, vincristine, and prednisone therapy (CHOP) for the rare patient with EATL presenting with persistent, severe gastrointestinal hemorrhage.