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

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 distrib...

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Autores principales: Khadadah, Fatima, Rupani, Natasha, Scott, Jordan, Trinkaus, Martina, Teitel, Jerome, Sholzberg, Michelle
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2022
Materias:
Case Reports
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9421994/
https://www.ncbi.nlm.nih.gov/pubmed/36051021
http://dx.doi.org/10.1002/jha2.486
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author Khadadah, Fatima
Rupani, Natasha
Scott, Jordan
Trinkaus, Martina
Teitel, Jerome
Sholzberg, Michelle
author_facet Khadadah, Fatima
Rupani, Natasha
Scott, Jordan
Trinkaus, Martina
Teitel, Jerome
Sholzberg, Michelle
author_sort Khadadah, Fatima
collection PubMed
description 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.
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spelling pubmed-94219942022-08-31 Lymphoma‐associated acquired von Willebrand syndrome responsive to splenectomy: A case report Khadadah, Fatima Rupani, Natasha Scott, Jordan Trinkaus, Martina Teitel, Jerome Sholzberg, Michelle EJHaem Case Reports 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. John Wiley and Sons Inc. 2022-05-31 /pmc/articles/PMC9421994/ /pubmed/36051021 http://dx.doi.org/10.1002/jha2.486 Text en © 2022 The Authors. eJHaem published by British Society for Haematology and John Wiley & Sons Ltd. https://creativecommons.org/licenses/by/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Reports
Khadadah, Fatima
Rupani, Natasha
Scott, Jordan
Trinkaus, Martina
Teitel, Jerome
Sholzberg, Michelle
Lymphoma‐associated acquired von Willebrand syndrome responsive to splenectomy: A case report
title Lymphoma‐associated acquired von Willebrand syndrome responsive to splenectomy: A case report
title_full Lymphoma‐associated acquired von Willebrand syndrome responsive to splenectomy: A case report
title_fullStr Lymphoma‐associated acquired von Willebrand syndrome responsive to splenectomy: A case report
title_full_unstemmed Lymphoma‐associated acquired von Willebrand syndrome responsive to splenectomy: A case report
title_short Lymphoma‐associated acquired von Willebrand syndrome responsive to splenectomy: A case report
title_sort lymphoma‐associated acquired von willebrand syndrome responsive to splenectomy: a case report
topic Case Reports
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9421994/
https://www.ncbi.nlm.nih.gov/pubmed/36051021
http://dx.doi.org/10.1002/jha2.486
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