新诊断慢性髓系白血病髓外T淋巴细胞急变患者的临床特点分析

Analysis of Clinical Characteristics in De novo Chronic Myelogenous Leukemia Patients with Extramedullary T-Lymphoblastic Blast Crisis

目的:报道1例新诊断慢性髓性白血病(chronic myelogenous leukemia,CML)髓外T淋巴细胞急变的患者的临床特点,以提高对该疾病的认识。方法:回顾性分析该患者的临床特征、诊疗方法,并对相关文献进行复习。结果:结合血常规、骨髓学、染色体和融合基因检查结果,考虑该患者为CML,患者淋巴结病理提示为T淋巴母细胞淋巴瘤;荧光原位杂交检测发现淋巴结病变中存在BCR-ABL融合信号,最终诊断为CML髓外T淋巴细胞急变。给予达沙替尼(140 mg/d)联合化疗,患者一直未达完全缓解。结论:新诊断的CML急变期患者相对少见,以CML髓外T淋巴细胞急变为特点并伴有附加染色体11q23的患者更为罕见。CML髓外T淋巴细胞急变的患者预后差,异基因造血干细胞移植或许是唯一有希望治愈的治疗方法。

Objective:To report the clinical characteristics in a case of extramedullary T-lymphoblastic blast crisis of de novo chronic myelogenous leukemia(CML)so as to improve the understanding of this disease.Methods:The clinical characteristics,diagnostic approach and treatment of the patient were retrospectively analyzed,and some related literatures were reviewed.Results:According to resuts of blood routine,bone marrow,chromosome and fusion gene tests,this patient was considered to be CML patient.The cervical lymph node biopsy indicated a T-cell lymphoblastic lymphoma(TLBL),and fluorescence in situ hybridization(FISH)analysis showed the BCR-ABL fusion gene within tumor cells of the patient’s lymphnodes,thus was confirmedly diagnosed as extramedullary T-lymphoblastic blast crisis of chronic myelogenous leukemia.Treatment with dasatinib 140 mg/d combined with chemotherapy was then initiated,while the patient never achieved a complete remission.Conclusion:De novo chronic myelogenous leukemia in blast crisis is infrequent presence,the cases of extramedullary T-lymphoblastic blast crisis of newly diagnosed CML with additional chromosome 11 q23 are extremely rare.And prognosis of these patients are poor,allogeneic hematopoietic stem cell transplantation maybe the only curable treatment.