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目的 研究骨髓纤维化合并门脉高压的特点。方法 对已确诊的 6例骨髓纤维化合并门脉高压患者进行胃镜、B超、CT、肝功能、核素心 /肝血流比、骨髓穿刺及活检等检查 ,并有 10例肝硬化患者作对照。结果 6例患者各项肝功能指标基本正常 ,白细胞与血小板数升高 ,骨髓象见纤维组织大量增生。胃镜 6例患者均有食管胃底静脉曲张 ,B超及 CT示肝弥漫性损害 ,均有门静脉增宽及脾大 ,核素心 /肝血流比测门脉压为 32± 2 cm H2 O。结论 骨髓纤维化可致门脉高压 ,其特点是肝功能基本正常 ,脾大而无脾亢之表现
Objective To study the characteristics of myelofibrosis combined with portal hypertension. Methods Six cases of myelofibrosis with portal hypertension were diagnosed by gastroscope, B ultrasound, CT, liver function, radionuclide heart / liver blood flow ratio, bone marrow aspiration and biopsy, and 10 patients with cirrhosis Control. Results All the 6 patients showed normal liver function indexes, leukocyte and platelet count increased, and bone marrow hyperplasia was seen in fibrous tissue. Gastroscopy in 6 patients with esophageal varices, B ultrasound and CT showed diffuse liver injury, both widened portal vein and splenomegaly, radionuclide ventricular blood pressure measured portal pressure was 32 ± 2 cm H2 O . Conclusion Myelofibrosis can cause portal hypertension, which is characterized by the basic normal liver function, splenomegaly without hypersplenism