裸细胞型ALK阳性间变性大细胞淋巴瘤1例并文献复习

ALK positive anaplastic large cell lymphoma of null-cell phenotype:A case report and literature review

回顾性分析陕西省人民医院收治的1例胸壁裸细胞型淋巴瘤激酶(anaplastic lymphoma kinase,ALK)阳性间变性大细胞淋巴瘤的临床病理特点并进行文献复习。该患者为51岁女性,因40 d前发现胸壁包块入院。超声示:左侧胸壁肌层内囊实性包块,大小约为8 cm×6 cm×3 cm;腹腔及后腹膜多发异常增大淋巴结,腹腔积液。行胸壁包块穿刺活体组织检查,小条穿刺组织镜下所见:纤维组织内有弥漫性小圆细胞浸润,细胞质嗜酸性,核呈圆形或卵圆形,部分核偏位,呈“马蹄形”。免疫组织化学染色示:细胞角蛋白、CD2、CD3、CD5、CD7、CD20、CD38、CD138、CD56及细胞周期蛋白D1均为阴性,ALK和CD30均为强阳性。T细胞抗原受体基因重排阳性。病理诊断为裸细胞型ALK阳性间变性大细胞淋巴瘤。该肿瘤非常罕见,其诊断需结合临床表现、组织学形态、免疫表型及分子遗传学改变进行综合判断,避免误诊。

Clinicopathological characteristics of a case of chest wall lymphoma kinase(ALK)-positive anaplastic large cell lymphoma of null-cell phenotype in the Shaanxi Provincial Peoples Hospital were retrospectively analyzed and literature review were performed.The patient,a 51-year-old female,was admitted due to the discovery of a chest wall mass found 40 days ago.Ultrasound revealed a solid cystic mass within the muscular layer of the left chest wall,measuring approximately 8 cm×6 cm×3 cm,along with multiple abnormally enlarged lymph nodes in the abdominal cavity and retroperitoneum,and peritoneal effusion.A needle biopsy of the chest wall mass was performed.Under the microscope,there were diffuse infiltration of small round cells in the fibrous tissue,with eosinophilic cytoplasm and round or ovoid nuclei,some of which were eccentric,presenting a“horseshoe shape”.Immunohistochemical staining showed strong positivity for CD30 and ALK,while cytokeratin,CD2,CD3,CD5,CD7,CD20,CD38,CD138,CD56 and cyclin D1 were negative.T-cell antigen receptor gene rearrangement was positive.The pathological diagnosis was ALK-positive anaplastic large cell lymphoma of null-cell phenotype.This tumour is extremely rare,and its diagnosis requires a comprehensive assessment considering clinical manifestations,histomorphology,immunophenotype and moleculargeneticchanges to avoid misdiagnosis.