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骨髓纤维化、脾肿大和门静脉高压症。

Myelofibrosis, splenomegaly, and portal hypertension.

作者信息

Jacobs P, Maze S, Tayob F, Harries-Jones E P

出版信息

Acta Haematol. 1985;74(1):45-8. doi: 10.1159/000206164.

DOI:10.1159/000206164
PMID:3934908
Abstract

A patient with chronic myelofibrosis and massive splenomegaly developed portal hypertension with haematemesis occurring from radiologically proven oesophageal varices. Transjugular liver biopsy showed only myeloid metaplasia, and radiological evaluation of the portal vascular system was undertaken to establish a diagnosis of hyperkinetic portal hypertension as a basis for therapeutic splenectomy. The alternative and rare situation of splenic and portal vein occlusion was demonstrated and therefore removal of the spleen was not an appropriate procedure for relief of portal hypertension. The variceal bleeding was successfully controlled with injection sclerotherapy.

摘要

一名患有慢性骨髓纤维化并伴有巨大脾肿大的患者出现门静脉高压,经放射学证实的食管静脉曲张导致呕血。经颈静脉肝活检仅显示髓外化生,对门静脉系统进行放射学评估以确诊高动力性门静脉高压,作为治疗性脾切除术的依据。结果显示存在脾脏和门静脉闭塞这种罕见的替代情况,因此切除脾脏并非缓解门静脉高压的合适方法。通过注射硬化疗法成功控制了静脉曲张出血。

相似文献

1
Myelofibrosis, splenomegaly, and portal hypertension.骨髓纤维化、脾肿大和门静脉高压症。
Acta Haematol. 1985;74(1):45-8. doi: 10.1159/000206164.
2
[Portal hypertension in the osteomyelofibrosis syndrome].
Dtsch Med Wochenschr. 1973 Aug;98(31):1463-71. doi: 10.1055/s-0028-1107056.
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Bleeding oesophageal varices with long term follow up.食管静脉曲张出血的长期随访
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Portal hypertension complicating myelofibrosis: reversal following splenectomy.门静脉高压并发骨髓纤维化:脾切除术后逆转
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6
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Leber Magen Darm. 1976 Dec;6(6):358-60.
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引用本文的文献

1
Case report: Primary myelofibrosis presenting with portal hypertension mimicking cirrhosis.病例报告:以酷似肝硬化的门静脉高压为表现的原发性骨髓纤维化。
Front Med (Lausanne). 2024 May 3;11:1375571. doi: 10.3389/fmed.2024.1375571. eCollection 2024.
2
Portal hypertension and myeloproliferative neoplasms: a relationship revealed.门静脉高压与骨髓增殖性肿瘤:一种已揭示的关系。
ISRN Hematol. 2013 Sep 16;2013:673781. doi: 10.1155/2013/673781. eCollection 2013.
3
Myelofibrosis and angiodysplasia of the colon: another manifestation of portal hypertension and massive splenomegaly?
骨髓纤维化与结肠血管发育异常:门静脉高压和巨脾的另一种表现形式?
J Clin Pathol. 2004 Sep;57(9):999-1000. doi: 10.1136/jcp.2003.015479.
4
Endoscopic injection sclerotherapy for esophageal variceal hemorrhage in a patient with idiopathic myelofibrosis.内镜下注射硬化疗法治疗特发性骨髓纤维化患者的食管静脉曲张出血
J Gastroenterol. 1996 Apr;31(2):260-2. doi: 10.1007/BF02389527.
5
Endoscopic injection sclerotherapy for esophageal variceal hemorrhage in myeloproliferative disorder: case report.骨髓增殖性疾病中内镜下注射硬化疗法治疗食管静脉曲张破裂出血:病例报告
J Gastroenterol. 1995 Apr;30(2):244-7. doi: 10.1007/BF02348672.