JAK2V617F positive early stage myeloproliferative disease (essential thrombocythemia) as the cause of portal vein thrombosis in two middle-aged women: therapeutic implications in view of the literature
| Autor: | R. H. van Rijssel, H. De Raeve, Jurgen Wegman, G. J. Hoogenboom, L. Scholten, J. J. Michiels, S. Commandeur |
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| Rok vydání: | 2007 |
| Předmět: |
Adult
medicine.medical_specialty Budd-Chiari Syndrome Thrombophilia Polymorphism Single Nucleotide Gastroenterology Mesenteric Veins Internal medicine medicine Humans Genetic Predisposition to Disease Platelet First episode Aspirin Hematology Portal Vein business.industry Essential thrombocythemia General Medicine Janus Kinase 2 medicine.disease Portal vein thrombosis Splanchnic vein thrombosis Splenic Vein Female Radiology business Thrombocythemia Essential medicine.drug |
| Zdroj: | Annals of hematology |
| ISSN: | 1432-0584 0939-5555 |
| DOI: | 10.1007/s00277-007-0351-1 |
| Popis: | The present study describes portal vein thrombosis (PVT) in two women as the first and single presenting symptom of latent or masked myeloproliferative disease (MPD). Essential thrombocythemia (ET) was suspected by a sustained increase in platelet count (400 x 10(9)/l) and slight splenomegaly on echogram. ET could be diagnosed by the presence of large platelet in peripheral blood smear, an increase in clustered large megakaryocytes in bone marrow smear and the presence of the JAK2(V617F) mutation. A subsequent biopsy specimen was consistent with the diagnosis of true ET. In patients with a first episode of splanchnic vein thrombosis (SVT), analysis of any venous thrombophilic risk factors as well as a JAK2(V617F) mutation status indicative for MPD is warranted. Administration of heparin followed by oral anticoagulation with vitamin K antagonists is the treatment of choice in patients with SVT. Anticoagulation therapy combined with low-dose aspirin and proper treatment of the MPD is recommended in patients with SVT associated with the JAK2(V617F) mutation. |
| Databáze: | OpenAIRE |
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