Lukie B E, Card R T
Can Med Assoc J. 1977 Oct 8;117(7):771-2.
Portal hypertension occurs in approximately 10% of patients with myelofibrosis. Increased portal blood flow secondary to splenomegaly has been proposed to explain its development. In a 60-year-old woman with proven myelofibrosis of 10 years' duration and gross splenomegaly, portal hypertension developed with esophageal varices and ascites. There was no demonstrable obstruction to portal blood flow. Following splenectomy the ascites and esophageal varices disappeared. Despite the presence of splenic myeloid metaplasia, splenectomy did not impair the patient's hematologic status. Portal hypertension complicating myelofibrosis has a poor prognosis, so careful attention should be given to its detection. Splenectomy may be preferable to portal-systemic shunting in the management of this complication.
门静脉高压症约在10%的骨髓纤维化患者中出现。继发于脾肿大的门静脉血流增加被认为是其发生的原因。一名60岁女性，确诊骨髓纤维化10年，伴有明显脾肿大，出现了门静脉高压症并伴有食管静脉曲张和腹水。未发现门静脉血流有明显梗阻。脾切除术后腹水和食管静脉曲张消失。尽管存在脾髓外化生，但脾切除术并未损害患者的血液学状态。骨髓纤维化合并门静脉高压症预后较差，因此应仔细关注其检测。在处理这种并发症时，脾切除术可能比门体分流术更可取。
