低蛋白性肥厚性胃病临床病理分析

Clinicopathological features of hypoproteinemic hypertrophic gastropathy

目的探讨低蛋白性肥厚性胃病(Menetrier病)临床病理学特征、免疫表型及鉴别诊断。方法回顾性分析3例Menetrier病的临床特征、组织学形态及免疫表型,并复习相关文献。结果 3例患者中,成人女性2例,小儿1例,临床表现均有腹痛及低蛋白血症,内窥镜显示胃窦及胃体胃壁明显增厚,表面高低不平,局部呈脑回样改变,局部结节状及息肉样改变,组织学显示小凹上皮增生明显,延伸并局部扩张,固有腺体萎缩,固有层水肿及急慢性炎细胞浸润。免疫表型:3例EGFR表达均增强,HP及CMV均阴性。3例患者出院后1例成人及小儿恢复良好,另1例成人病情反复,3年后因其他相关疾病死亡。结论Menetrier病为罕见疾病,临床表现及检查无特异性,易误诊为其它疾病,需结合组织学形态并排除其他鉴别诊断,必要时行内镜黏膜切除来明确诊断。

Purpose To explore the clinical and pathological features and differential diagnosis of hypoproteinemie hypertrophic gastropathy （ Menetrier disease）. Methods Clinical features, histological morphology and immunohistochemieal results of 3 cases of Menettler disease were analyzed and the related literatures were reviewed as well. Results Three cases of Menetrier disease, two adult women and a child, presented with abdominal pain and hypoalbuminemia. Endoscopy results revealed marked thickening and uneven surface of wall of gastric antrum and body with local gyri, nodular and polypoid changes. Histological examination showed significantly hyperplasia, extension and expansion in foveola epithelia. Inherent glands were atrophy along with lamina propria edema and acute or chronic inflammatory cells infiltration in lamina propria. Immunohistoehemieal staining showed the expression of EGFR and Ki-67 was enhanced, while HP and CMV stains were negative. One of the adult and the child patients were healed, while the other adult patient recurred frequently and died after 3 years due to other related diseases. Conclusion Menetrier disease is a rare lesion, which has no specific clinical manifestations and laboratory examinations. The diagnosis is much relied on histology and ruling out other possible differential diagnosis. Both pathological and clinical features are needed for definitive diagnosis.