식도정맥류 출혈과 다발성 혈전증으로 발견된 본태성 혈소판 증다증 1예
A Case of Essential Thrombocythemia Presenting as Esophageal Varix Bleeding and Multiple Thrombosis
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Essential thrombocythemia (ET), a subcategory of chronic myeloproliferative disorder, is characterized by absolute thrombocytosis due to excessive clonal proliferation of platelets, hyperaggregability of platelets, and increased incidence of thrombosis and hemorrhage. We consider a diagnosis of ET when an unexplained and persistent thrombocytosis is observed. It is difficult to consider ET first when we meet a patient with esophageal varix bleeding or unusual multiple thromboses like mesenteric vein, splenic vein, and portal vein. This article reports a patient who presented initially with esophageal varix bleeding and unusual multiple thromboses, thereafter, she was diagnosed with ET after testing positive for the Janus Tyrosine Kinase 2 (JAK2) V617F mutation. In conclusion, in patients with varix bleeding and unusual multiple thromboses, myeloproliferative disorders like essential thrombocythemia should be considered as a potential cause and testing for the JAK2 mutation is warranted.
Abstract
Essential thrombocythemia (ET), a subcategory of chronic myeloproliferative disorder, is characterized by absolute thrombocytosis due to excessive clonal proliferation of platelets, hyperaggregability of platelets, and increased incidence of thrombosis and hemorrhage. We consider a diagnosis of ET when an unexplained and persistent thrombocytosis is observed. It is difficult to consider ET first when we meet a patient with esophageal varix bleeding or unusual multiple thromboses like mesenteric vein, splenic vein, and portal vein. This article reports a patient who presented initially with esophageal varix bleeding and unusual multiple thromboses, thereafter, she was diagnosed with ET after testing positive for the Janus Tyrosine Kinase 2 (JAK2) V617F mutation. In conclusion, in patients with varix bleeding and unusual multiple thromboses, myeloproliferative disorders like essential thrombocythemia should be considered as a potential cause and testing for the JAK2 mutation is warranted.
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