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门静脉高压作为骨髓增殖性疾病的首发特征。诊断及治疗困境。

Portal hypertension as presenting feature of a myeloproliferative disorder. Diagnosis and therapeutic dilemmas.

作者信息

Muller E W, De Wolf J T, Haagsma E B

机构信息

Dept. of Internal Medicine, University Hospital Groningen, The Netherlands.

出版信息

Scand J Gastroenterol Suppl. 1993;200:74-9. doi: 10.3109/00365529309101580.

DOI:10.3109/00365529309101580
PMID:8016576
Abstract

In some patients presenting with complications of portal hypertension, thrombosis of hepatic or portal veins is identified as the cause. Hepatic or portal vein thrombosis may be secondary to recognized etiologies like infection or malignancy. When no etiology for the thrombosis is found, it is likely that a 'latent' myeloproliferative disorder (MPD) is the underlying abnormality. We present seven patients referred to us between 1988 and 1993 with complications of portal hypertension due to hepatic or portal vein thrombosis, in whom a 'latent', and in one patient overt, MPD was identified as the underlying disorder. Problems relating to the diagnosis of (latent) MPD in this subset of patients are discussed. The importance of in vitro 'endogenous' erythroid colony formation indicating the presence of MPD is emphasized. Also, a therapeutic strategy, with special emphasis on anticoagulation therapy, is suggested.

摘要

在一些出现门静脉高压并发症的患者中,肝静脉或门静脉血栓形成被确定为病因。肝静脉或门静脉血栓形成可能继发于感染或恶性肿瘤等已知病因。当未发现血栓形成的病因时,很可能潜在的骨髓增殖性疾病(MPD)是潜在的异常情况。我们报告了1988年至1993年间转诊至我院的7例因肝静脉或门静脉血栓形成导致门静脉高压并发症的患者,其中发现潜在的MPD,1例患者为明显的MPD,被确定为潜在疾病。讨论了在这部分患者中诊断(潜在)MPD的相关问题。强调了体外“内源性”红系集落形成表明MPD存在的重要性。此外,还提出了一种治疗策略,特别强调抗凝治疗。

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Portal hypertension as presenting feature of a myeloproliferative disorder. Diagnosis and therapeutic dilemmas.门静脉高压作为骨髓增殖性疾病的首发特征。诊断及治疗困境。
Scand J Gastroenterol Suppl. 1993;200:74-9. doi: 10.3109/00365529309101580.
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[Myeloproliferative diseases as causative agents of portal and hepatic veins thrombosis].[骨髓增殖性疾病作为门静脉和肝静脉血栓形成的致病因素]
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In vitro colony culture and chromosomal studies in hepatic and portal vein thrombosis--possible evidence of an occult myeloproliferative state.肝门静脉血栓形成的体外集落培养及染色体研究——隐匿性骨髓增殖状态的可能证据
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Treatment of gastric varices with partial splenic embolization in a patient with portal vein thrombosis and a myeloproliferative disorder.门静脉血栓形成合并骨髓增殖性疾病患者采用部分脾栓塞术治疗胃静脉曲张
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