骨髓纤维化导致的食道-胃底静脉破裂出血讨论  

Discussion of myelofibrosis leading to the rupture of the esophagus-gastric fundus vein

作　　者：张延涛 甘贻波 唐源 黄初军 ZHANG Yantao;GAN Yibo;TANG Yuan;HUANG Chujun(Department of Gastroenterology,Qujing First People's Hospital,Qujing,Yunnan,655000,China)

机构地区：[1]曲靖市第一人民医院消化内科,云南曲靖655000

出　　处：《当代医学》2021年第33期64-65,共2页Contemporary Medicine

摘　　要：目的充分认识少见疾病导致的食道-胃底静脉曲张破裂出血。方法回顾性分析本院2018年收治的32例食道-胃底静脉曲张破裂出血患者的临床资料。分析其中2例原发性骨髓纤维化患者的临床资料与普通肝硬化失代偿期食道-胃底静脉曲张破裂出血患者的资料差异。结果无创肝纤维显示30例肝硬化失代偿期患者肝脏硬度明显增加,而2例原发性骨髓纤维化患者肝脏硬度基本正常。32例患者中血清白蛋白、总胆红素、转氨酶数值及血小板数,肝硬化失代偿期导致的食道-胃底静脉出血患者下降幅度明显大于原发性骨髓纤维化患者。结论血液系统疾病特别是骨髓增殖性疾病如骨髓纤维化导致的门静脉高压并不少见,临床上遇到食道、胃底静脉曲张,肝损伤轻,脾脏增大明显又无明显肝硬化的典型表现者,需考虑到罕见的导致门静脉高压的疾病,如区域性门脉高压症、门静脉血栓、布加综合征等。消化专科更为少见的骨髓增殖性疾病易被忽视,通过上述病例的分享以期减少误诊、误治,给患者更加准确的诊治。Objective To fully understand the rupture and hemorrhage of esophagus-gastric varicose veins caused by rare diseases.Methods Clinical data of 32 patients with esophagogastric varicose vein rupture and hemorrhage admitted to our hospital in 2018 were retrospectively analyzed.The differences between the clinical data of two patients with primary myelofibrosis and those of the patients with esophageal varicose vein rupture and hemorrhage in decompensated stage of common cirrhosis were analyzed.Results The hardness of 30 patients with decompensated cirrhosis was significantly increased,while the hardness of 2 patients with primary myelofibrosis was basically normal.Among the 32 patients,serum albumin,total bilirubin,transaminase value and platelet count were significantly lower in patients with esophageal and gastric fundus venous bleeding caused by decompensated cirrhosis than in patients with primary myelofibrosis.Conclusion Blood system diseases,especially bone marrow hyperplastic diseases such as bone marrow fibrosis caused by portal hypertension is not uncommon,esophageal and gastric varices clinically,liver damage,spleen increased significantly and no obvious typical behavior,want to consider some rare cause of portal hypertension disease,such as regional portal hypertension,portal vein thrombosis,cloth and syndrome,etc.Bone marrow proliferative diseases,which are less common in digestive specialties,are more likely to be overlooked.Through the sharing of the above cases,the author hopes to reduce misdiagnosis and treatment and give more accurate diagnosis and treatment to the patients.

关 键 词：原发性骨髓纤维化 食道-胃底静脉破裂出血 

分 类 号：R575[医药卫生—消化系统]