以纵隔肿块为表现的慢性粒细胞白血病髓外急变  

A Case of Extramedullary Blast Crisis of CML Presented as a Large Mediastinum Lump

作　　者：林志美[1,2] 朱焕玲[1] 饶进[2] 殷莉[2] 李世云[2] 

机构地区：[1]四川大学华西医院血液内科,成都610044 [2]成都大学附属医院血液内科,成都610081

出　　处：《临床误诊误治》2014年第12期28-31,共4页Clinical Misdiagnosis & Mistherapy

摘　　要：目的探讨慢性粒细胞白血病髓外急变(extramedullary blast crisis of chronic myelogenous leukemia,CML-EBC)的诊断及治疗要点。方法对1例CML-EBC的临床资料进行回顾性分析并复习相关文献。结果本例因确诊慢性粒细胞白血病3年,胸背痛伴呼吸困难2个月入院。行胸部CT扫描示胸腹主动脉旁椎前梭形软组织密度影;复查骨髓涂片+骨髓流式细胞检测符合慢性粒细胞白血病(慢性期);行胸腔镜下纵隔病变活检及免疫组织化学检查,诊断考虑恶性肿瘤浸润,淋巴造血系来源可能;组织病理切片荧光原位杂交(FISH)技术分析示BCR/ABL融合基因阳性。明确诊断为慢性粒细胞白血病髓外B淋巴细胞急性变,予伊马替尼等药物化疗后病情好转出院,继续予伊马替尼维持治疗。结论 CML-EBC易误诊为慢性粒细胞白血病并淋巴瘤,临床应注意鉴别,治疗可选用化疗、局部放疗、造血干细胞移植等综合措施。Objective To explore the diagnosis, differential diagnosis and therapy of extramedullary blast crisis of chronic myelogenous leukemia （ CML-EBC） . Methods A case of extramedullary blast crisis of CML presented as a large me-diastinum lump was reported and the related literature was reviewed. Results With diagnosis of chronic myelogenous leuke-mia for 3 years, this patient was readmitted for chest pain associated with dyspnea. The chest CT scan showed that there was a spindle soft tissue density by the thoracic and abdominal aorta. Bone marrow smear and fluid cytology confirmed chronic phase of the chronic myelogenous leukemia. Mediastinal lesions biopsy under thoracoscope, and immunohistochemistry fea-tures suggested malignant tumor infiltration from the lymphatic hematopoietic system. Chronic myelogenous leukaemia-extr-amedullary blast crisis from B lymphocytes was confirmed. After the treatment of imatinib, the patient condition was improved and was discharged from hospital. The patient continued to take the imatinib for maintenance treatment. Conclusion CML-EBC presented as a local lump tends to be misdiagnosed as CML with lymphoma if tumour cells expressing lymphocyte markers and the diagnosis relied on the fusion signals between BCR and ABL genes, which were identified in the biopsy specimens by FISH. Special attention to the differential diagnosis should be paid. Combined therapy of tyrosine kinase inhibitors and chemo-therapy, radiotherapy or stem cell transplantation is an optional choice to in the treatment CML-EBC.

关 键 词：白血病 髓样 白血病 髓样 加速期 误诊 淋巴瘤 原位杂交 荧光 

分 类 号：R733.72[医药卫生—肿瘤]