特殊T淋巴母细胞淋巴瘤一例诊治并文献复习

Diagnosis and treatment of special T-lymphoblast lymphoma： report of one case and review of literature

目的 探讨伴嗜酸性粒细胞增高的髓淋系肿瘤（FIP1L1-PDGFRα重排型）的正确诊断和治疗.方法 报道1例伴嗜酸性粒细胞增高的髓淋系肿瘤（FIP1L1-PDGFRα重排型）患者的诊治过程,并进行相关文献复习.结果 该患者根据淋巴结病理诊断为典型的T淋巴母细胞淋巴瘤,经全面分析和检查正确诊断为伴嗜酸性粒细胞增高的髓淋系肿瘤（FIP1L1-PDGFRα重排型）,并经低剂量伊马替尼治疗获得快速深度缓解.结论 伴嗜酸性粒细胞增高的髓淋系肿瘤（FIP1L1-PDGFRα重排型）为罕见的血液肿瘤,虽然病理诊断是淋巴瘤诊断的金标准,但有时需要综合考虑,全面分析临床情况,正确看待病理结果,避免误诊、误治.

Objective To investigate the correct diagnosis and treatment of myeloid and lymophoid neoplasms with eosinophilia and the FIP1L1-PDGFR fusion gene. Methods A case of patient who was diagnosed as myeloid and lymophoid neoplasms with eosinophilia and the FIP1L1-PDGFR fusion gene was reported, and the literature was reviewed. Results The patient was diagnosed as typical T-lymphoblast lymphoma （T-LBL） by the lymph node pathologic diagnosis, while the diagnosis of myeloid and lymophoid neoplasms with eosinophilia and the FIP1L1-PDGFR fusion gene was made correctly by the whole examination and analysis. The patient acquired deep complete remission quickly after taking the low dose of imatinib. Conclusions Myeloid and lymophoid neoplasms with eosinophilia and the FIP1L1-PDGFR fusion gene are a rare hematologic tumor. Though pathological diagnosis is the golden standard for lymphoma, sometimes the other factors should be taken into consideration and make an overall analysis of clinical picture and a correct view of the pathological diagnosis, which could avoid the misdiagnosis and improper treatment.