Lymphoma-associated acquired von Willebrand syndrome responsive to splenectomy: A case report

eJHaem

article

Fatima Khadadah¹  Natasha Rupani²  Jordan Scott²  Martina Trinkaus³  Jerome Teitel³  Michelle Sholzberg³ 

[1] Division of Hematology, Kuwait Cancer Control Centre;Division of Hematology, Department of Medicine, University of Toronto;Division of Hematology/Oncology, Departments of Medicine and Laboratory Medicine & Pathobiology, St. Michael's Hospital, University of Toronto

关键词: acquired von Willebrand syndrome;    case report;    hemostasis;   

DOI  :  10.1002/jha2.486

来源: Wiley

【 摘 要 】

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.

【 授权许可】

Unknown