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血液系统疾病中的肝脏结节性再生性增生:对闭塞性门静脉病的一种可能反应。9例病例的形态计量学研究及发病机制假说

Nodular regenerative hyperplasia of the liver in hematologic disorders: a possible response to obliterative portal venopathy. A morphometric study of nine cases with an hypothesis on the pathogenesis.

作者信息

Wanless I R, Godwin T A, Allen F, Feder A

出版信息

Medicine (Baltimore). 1980 Sep;59(5):367-79.

PMID:7432153
Abstract

Nodular regenerative hyperplasia was found in nine patients who had hematological disease including polycythemia vera, agnogenic myeloid metaplasia, primary thrombocythemia, rheumatoid arthritis with thrombocytosis, multiple myeloma, and erythrocytosis associated with polycystic renal disease. Portal hypertension was suspected in three and features of hypersplenism were present in four. 2. Nodular regenerative hyperplasia occurred in livers which had widespread obliteration of portal vein radicals (obliterative portal venopathy). Morphometric analysis indicated that the portal vein lesions were predominately located in veins up to 0.2 mm in diameter and were significantly more frequent than similar lesions occurring in elderly persons. 3. The following pathogenesis of nodular regenerative hyperplasia is proposed: Thrombi, perhaps largely composed of platelet aggregates formed in the portal venous circulation or spleen, embolize to the liver and results in obliterative vascular lesions. Atrophy and regenerative nodule formation occur in response to the interruption of the portal blood supply.

摘要

在9例患有血液系统疾病的患者中发现了结节性再生性增生,这些疾病包括真性红细胞增多症、原发性骨髓纤维化、原发性血小板增多症、伴有血小板增多症的类风湿性关节炎、多发性骨髓瘤以及与多囊肾相关的红细胞增多症。3例患者疑似存在门静脉高压,4例有脾功能亢进的表现。2. 结节性再生性增生发生于门静脉分支广泛闭塞(闭塞性门静脉病)的肝脏。形态计量分析表明,门静脉病变主要位于直径达0.2mm的静脉,且比老年人中出现的类似病变更为常见。3. 提出了结节性再生性增生的以下发病机制:血栓,可能主要由在门静脉循环或脾脏中形成的血小板聚集体组成,栓塞至肝脏并导致闭塞性血管病变。门静脉血液供应中断后会出现萎缩和再生结节形成。

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Nodular regenerative hyperplasia of the liver in hematologic disorders: a possible response to obliterative portal venopathy. A morphometric study of nine cases with an hypothesis on the pathogenesis.血液系统疾病中的肝脏结节性再生性增生:对闭塞性门静脉病的一种可能反应。9例病例的形态计量学研究及发病机制假说
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