CASE REPORTSAcquired von Willebrand syndrome in a patient with small lymphocytic lymphoma and Sjögren's syndrome which associated condition should be prioritized?Pardos-Gea, Joséa; Martínez, Fernandab; Abrisqueta, Pauc; Santamaría, Amparob; Bosch, FrancesccAuthor Information aAutoimmune Diseases Unit, Department of Internal Medicine bHaemophilia Unit, Department of Hematology cDepartment of Hematology and Vall d’Hebrón Institute of Oncology (VHIO), Vall d’Hebrón University Hospital, Barcelona, Spain Correspondence to José Pardos-Gea, MD, PhD, Autoimmune Diseases Unit, Department of Internal Medicine, Vall d’Hebrón University Hospital, Passeig de la Vall Hebrón 115-119, 08035 Barcelona, Spain E-mail: [email protected] Received 22 January, 2019 Revised 23 April, 2019 Accepted 24 April, 2019 Blood Coagulation & Fibrinolysis: July 2019 - Volume 30 - Issue 5 - p 239-242 doi: 10.1097/MBC.0000000000000814 Buy Metrics Abstract Acquired von Willebrand syndrome (AVWS) is a rare hemorrhagic condition that poses both a diagnostic and a therapeutic challenge. Here we report a singular case of AVWS with two associated conditions, small lymphocytic lymphoma (SLL) and Sjögren's syndrome. The patient presented with recurrent and severe digestive bleeding that forced us to raise a curative attempt of AVWS. A first immunosuppressive therapy with immunoglobulins was unsuccessful and it was later decided to treat lymphoproliferative entity with bendamustine and rituximab effectively achieving SLL and AVWS remission. On the basis of our case and through literature review, we discuss potential strategies to achieve AVWS remission when it appears in the setting of several causative associated conditions. Copyright © 2019 YEAR Wolters Kluwer Health, Inc. All rights reserved.