继发于慢性髓系白血病的食道粒细胞肉瘤患者的临床分析

Clinical Analysis of Patient with Esophageal Granulocytic Sarcoma Derived from Chronic Myelocytic Leukemia

目的:探讨食道粒细胞肉瘤的临床特点和诊治方法。方法:对1例继发于慢性髓系白血病(CML)的食道粒细胞肉瘤的临床表现、诊断及治疗进行回顾性分析。结果:老年女性72岁,因胸骨后疼痛伴乏力,在当地医院行胃镜发现食管距门齿28 cm处可见1.0 cm×0.5 cm欠规则隆起,表面覆糜烂及少量鲜血;病理结果提示,见异型的淋巴样细胞浸润,胞质红染,另见较多的中性粒细胞浸润;同时发现外周血白细胞增高,转诊我院。免疫组化标记结果显示,AE1/AE3、CK5/6与p63显示鳞状上皮(+);异型的淋巴样细胞CD20^-、CD23^-、CD3^-、CD5^-、CD79a^-、MP0^+、Ki-67^+(80%);FISH检查提示BCR/ABL^+。骨髓细胞形态学、骨髓血BCR/ABL以及染色体核型分析等检查确诊为慢性髓系白血病,结合食道粒细胞肉瘤诊断为慢性粒细胞白血病(急变期)合并食道粒细胞肉瘤。给予甲磺酸伊马替尼0.6 g/d口服治疗,患者门诊随诊。结论:食道粒细胞肉瘤临床罕见,临床症状无特异性,对于白血病患者出现食道症状需要用胃镜进行筛查,对于继发性慢性髓系白血病的食道粒细胞肉瘤需要按照慢性髓系白血病急性变进行治疗。

Objective:To investigate the diagnosis and treatment of esophageal granulocytic sarcoma derived from chronic myelocytic leukemia(CML).Methods:The clinical manifestations,diagnosis and treatment of 1 case of esophageal granulocytic sarcoma secondary from chronic myelocytic leukemia were retrospectively analyzed and the related literature was reviewed.Results:The patient was a 72-year-old woman with poststernal pain accompanied by general weakness.Gastroscopy was performed in a local hospital.At the same time,the increase of peripheral blood leucocytes was obvious.Under gastroscopy,1.0 cm×0.5 cm irregular protuberance was found at 28 cm from the esophagus to the incisor teeth,and the surface was covered with erosion and a small amount of blood.Pathological results showed that heterotypic lymphoid cell infiltration,cytoplasmic red staining and more neutrophils were seen.Immunohistochemical staining results showed that AE1/AE3,CK5/6 and p63 displayed squamous epithelium(+);atypical lymphoid cells CD20^-,CD23^-,CD3^-,CD5^-,CD79 a^-,MP0^+,Ki-67^+(80%)were observed;FISH examination showed positive expression of BCR/ABL.The patient was further examined on myelogran and was diagnosed as chronic myelocytic leukemia with esophageal granulocytic sarcoma.Imatinib was given orally and the patient was followed up in the clinic.Conclusion:Esophageal granulocytic sarcoma is rare in clinic,its clinical symptoms are not specific.Gastroscopy should be routinely screened for esophageal discomfort,and the esophageal granulocytic sarcoma derived from CML is treated according to the therapeutic regimen of the acute transformation of chronic myelocytic leukemia.