Lymphoma‐associated acquired von Willebrand syndrome responsive to splenectomy: A case report
Fatima Khadadah,
Natasha Rupani,
Jordan Scott,
Martina Trinkaus,
Jerome Teitel,
Michelle Sholzberg
Affiliations
Fatima Khadadah
Division of Hematology Kuwait Cancer Control Centre Shuwaikh Kuwait
Natasha Rupani
Division of Hematology Department of Medicine University of Toronto Toronto Canada
Jordan Scott
Division of Hematology Department of Medicine University of Toronto Toronto Canada
Martina Trinkaus
Division of Hematology/Oncology, Departments of Medicine and Laboratory Medicine & Pathobiology St. Michael's Hospital, University of Toronto Toronto Canada
Jerome Teitel
Division of Hematology/Oncology, Departments of Medicine and Laboratory Medicine & Pathobiology St. Michael's Hospital, University of Toronto Toronto Canada
Michelle Sholzberg
Division of Hematology/Oncology, Departments of Medicine and Laboratory Medicine & Pathobiology St. Michael's Hospital, University of Toronto Toronto Canada
Abstract A previously healthy 33‐year‐old female presented with a large hematoma over her right knee after kneeling. She was found to have pancytopenia and massive splenomegaly. Von Willebrand Factor (VWF) antigen level was 0.38 units/ml, ristocetin cofactor activity 0.13 units/ml, and VWF multimeric distribution was normal. Bone marrow examination revealed an indolent B‐cell lymphoma. Diagnosis was consistent with acquired von Willebrand syndrome as an autoimmune epiphenomenon of a lymphoma. Diagnostic and therapeutic splenectomy under hemostatic coverage was performed. VWF antigen levels and activities immediately normalized postoperatively and remained within the normal range several months later. Splenic pathology confirmed hairy cell leukemia with a BRAF mutation.
