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门静脉高压与骨髓增殖性肿瘤:一种已揭示的关系。

Portal hypertension and myeloproliferative neoplasms: a relationship revealed.

作者信息

Toros Ahmet Burak, Gokcay Serkan, Cetin Guven, Ar Muhlis Cem, Karagoz Yesim, Kesici Besir

机构信息

Liv Ulus Hospital, Department of Gastroenterohepatology, 34347 Istanbul, Turkey.

出版信息

ISRN Hematol. 2013 Sep 16;2013:673781. doi: 10.1155/2013/673781. eCollection 2013.

Abstract

Background/Objectives. Patients with myeloproliferative neoplasms have a well-established increased risk of thrombosis. Many trials report identification of an underlying myeloproliferative neoplasm by investigation of the patients developing portal hypertensive esophagus and/or fundus variceal hemorrhage in the absence of any known etiology. This trial was designed to investigate the association between myeloproliferative neoplasms and portal hypertension and to detect the frequency of portal hypertension development in this subset of patients. Methodology. Twenty-nine patients previously diagnosed with polycythemia vera, essential thrombocytopenia, and primary myelofibrosis, who were under followup at the hematology outpatient clinic of our hospital, were included in the trial. Results. In our trial, we detected portal hypertension in 13.8% of the patients (n = 4), as a finding that was similar to those obtained in other studies performed to date. Conclusions. Considering the fact that diagnosis of myeloproliferative neoplasms usually takes a long time, treatment should be started (while, on the other hand, assessing the investigational and therapeutical choices for the complications) right after the bone marrow biopsy or cytogenetic studies required for establishing the final diagnosis have been performed.

摘要

背景/目的。骨髓增殖性肿瘤患者发生血栓形成的风险显著增加,这一点已得到充分证实。许多试验报告称,在无任何已知病因的情况下,通过对出现门静脉高压性食管和/或胃底静脉曲张出血的患者进行调查,可发现潜在的骨髓增殖性肿瘤。本试验旨在研究骨髓增殖性肿瘤与门静脉高压之间的关联,并检测该类患者门静脉高压的发生频率。方法。本试验纳入了29例先前诊断为真性红细胞增多症、原发性血小板增多症和原发性骨髓纤维化的患者,这些患者正在我院血液科门诊接受随访。结果。在我们的试验中,我们检测到13.8%的患者(n = 4)存在门静脉高压,这一结果与迄今进行的其他研究结果相似。结论。鉴于骨髓增殖性肿瘤的诊断通常需要很长时间,在进行了确立最终诊断所需的骨髓活检或细胞遗传学研究后,应立即开始治疗(另一方面,同时评估针对并发症的检查和治疗选择)。

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