CAJ | 학술논문

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。
목적：탐토만성립세포백혈병수외급변( extramedullary blast crisis of chronic myelogenous leukemia, CML-EBC)적진단급치료요점。방법대1례CML-EBC 적림상자료진행회고성분석병복습상관문헌。결과본례인학진만성립세포백혈병3년,흉배통반호흡곤난2개월입원。행흉부CT소묘시흉복주동맥방추전사형연조직밀도영；복사골수도편+골수류식세포검측부합만성립세포백혈병(만성기)；행흉강경하종격병변활검급면역조직화학검사,진단고필악성종류침윤,림파조혈계래원가능；조직병리절편형광원위잡교( FISH)기술분석시BCR/ABL융합기인양성。명학진단위만성립세포백혈병수외B림파세포급성변,여이마체니등약물화료후병정호전출원,계속여이마체니유지치료。결론 CML-EBC역오진위만성립세포백혈병병림파류,림상응주의감별,치료가선용화료、국부방료、조혈간세포이식등종합조시。
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.