Files and links (2)
Published, Version of Record (VoR)Open Access (License Unspecified), Open
Journal article
Triple-Negative Essential Thrombocythemia Complicated by Thrombosis and Acquired von Willebrand Disease in a Young Man
The American journal of case reports, v 21, pp 1-5
23 Aug 2020
PMID: 32829377
Featured in Collection : UN Sustainable Development Goals @ Drexel
Abstract
Objective: Rare co-existance of disease or pathology
Background: Essential thrombocythemia (ET) is a type of myeloproliferative neoplasm (MPN) characterized by sustained thrombocytosis in peripheral blood. Patients typically have gene mutations like JAK2V617F, CALR, and MPLW515L/K. This report describes a young man with ET without any of the above mutations who had paradoxical bleeding due to acquired Von-Willebrand disease.
Case Report: A young man with a medical history of thrombocytosis on aspirin presented with acute chest pain and was found to have had a myocardial infarction. Emergency cardiac catheterization revealed a thrombotic occlusion of the left anterior descending (LAD) artery and the right posteriolateral system, with an ejection fraction of 25%. He underwent thrombectomy and balloon angioplasty with LAD stenting, and an Impella 2.5 was inserted due to severe left ventricular dysfunction with akinesia. Aspirin and ticagrelor were administered, but the patient later experienced postoperative bleeding from the site of the Impella device. The bleeding was attributed to acquired Von-Willebrand disease secondary to ET. Emergency plateletpheresis was recommended. Further workup demonstrated that he was triple-negative for JAK2, MPL, and CALR gene mutations.
Conclusions: The paradoxical bleeding resulting from acquired Von-Willebrand disease was likely an entirely separate entity from the hyper-thrombotic state expected from ET. Careful assessment of clinical symptoms and laboratory markers, in addition to a high degree of suspicion, are needed to diagnose acquired Von-Willebrand disease as a complication of ET.
Metrics
Details
- Title
- Triple-Negative Essential Thrombocythemia Complicated by Thrombosis and Acquired von Willebrand Disease in a Young Man
- Creators
- Tejaswi Kanderi - UPMC Central PaMax Puthenpura - Drexel Univ, Coll Med, Dept Internal Med, Philadelphia, PA 19104 USAIsha Shrimanker - UPMC Central PaFnu Sapna - UPMC Central PaScott C. Felter - University of Pittsburgh Medical Center
- Publication Details
- The American journal of case reports, v 21, pp 1-5
- Publisher
- Int Scientific Information, Inc
- Number of pages
- 5
- Resource Type
- Journal article
- Language
- English
- Academic Unit
- General Internal Medicine
- Web of Science ID
- WOS:000563793300001
- Scopus ID
- 2-s2.0-85089794612
- Other Identifier
- 991021860777204721
UN Sustainable Development Goals (SDGs)
This publication has contributed to the advancement of the following goals:
InCites Highlights
Data related to this publication, from InCites Benchmarking & Analytics tool:
- Web of Science research areas
- Hematology