Valla D, Casadevall N, Huisse M G, Tulliez M, Grange J D, Muller O, Binda T, Varet B, Rueff B, Benhamou J P
Service d'Hépatologie, Hôpital Beaujon, Clichy, France.
Gastroenterology. 1988 Apr;94(4):1063-9. doi: 10.1016/0016-5085(88)90567-7.
In a prospective study of 33 adults with portal vein thrombosis unrelated to a liver tumor, we have assessed the prevalence of primary myeloproliferative disorders using conventional criteria and cultures of bone marrow progenitor cells. A primary myeloproliferative disorder was documented in 14 patients investigated at the time of recognition of portal vein thrombosis. Among these 14 patients, the main clue to the presence of the myeloproliferative disorder was (a) the observation of suggestive abnormalities of peripheral blood cell counts in 4 patients; (b) characteristic findings at bone marrow biopsy or determination of total red cell volume in 3 patients; and (c) formation of "spontaneous" erythroid colonies in cultures of bone marrow progenitor cells in erythropoietin-poor medium in 7 patients. In 2 other patients, agnogenic myeloid metaplasia with myelosclerosis of apparently recent onset developed 5 yr after recognition of portal vein thrombosis. In conclusion, primary myeloproliferative disorders--in a full-blown or latent form, or at an early stage--are a major cause of portal vein thrombosis.
在一项针对33例与肝肿瘤无关的门静脉血栓形成的成年患者的前瞻性研究中，我们使用传统标准和骨髓祖细胞培养评估了原发性骨髓增殖性疾病的患病率。在确诊门静脉血栓形成时接受检查的14例患者中记录到原发性骨髓增殖性疾病。在这14例患者中，骨髓增殖性疾病存在的主要线索是：（a）4例患者外周血细胞计数观察到提示性异常；（b）3例患者骨髓活检或总红细胞体积测定有特征性发现；（c）7例患者在低促红细胞生成素培养基中骨髓祖细胞培养形成“自发”红系集落。另外2例患者在确诊门静脉血栓形成5年后发生了近期明显起病的原因不明的髓样化生伴骨髓硬化。总之，原发性骨髓增殖性疾病——无论是显性、隐性还是早期——都是门静脉血栓形成的主要原因。
