Wei Jia, Huang Mei, Wang Ying, Zhou Jianfeng
Department of Hematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Onkologie. 2013;36(3):119-22. doi: 10.1159/000348681. Epub 2013 Feb 25.
Chronic myeloid leukemia (CML) is usually diagnosed in the chronic phase. An extramedullary blast crisis mimicking a T-cell lymphoma is a rare finding.
A 35-year-old man presented with multiple lymphadenopathy 2 months after diagnosis of Philadelphia chromosome (Ph)-positive CML in the chronic phase. Cervical lymph node biopsy later indicated an extramedullary blast crisis resembling T-cell lymphoblastic lymphoma (TLBL). The tumor mass was composed of primitive lymphoid cells expressing terminal deoxynucleotidyl transferase (TdT), CD3, CD43, CD5, CD99, and Bcl-2. Although the pathological diagnosis, confirmed by 2 independent pathological centers, was more typical of TLBL, fluorescence in situ hybridization (FISH) analysis showed the bcr-abl fusion gene within the blastic tumor cells. The FISH finding confirmed that the mass represented an extramedullary, immature blastic transformation of CML rather than a de novo T-cell lymphoma.
The diagnosis of de novo TLBL should be suspected before excluding the extramedullary blast phase of CML.
慢性髓性白血病（CML）通常在慢性期被诊断出来。模仿T细胞淋巴瘤的髓外原始细胞危象是一种罕见的发现。
一名35岁男性在慢性期被诊断为费城染色体（Ph）阳性CML 2个月后出现多处淋巴结病。随后的颈部淋巴结活检显示为类似于T细胞淋巴母细胞淋巴瘤（TLBL）的髓外原始细胞危象。肿瘤块由表达末端脱氧核苷酸转移酶（TdT）、CD3、CD43、CD5、CD99和Bcl-2的原始淋巴细胞组成。尽管经2个独立病理中心确认的病理诊断更符合TLBL，但荧光原位杂交（FISH）分析显示原始肿瘤细胞内存在bcr-abl融合基因。FISH结果证实该肿块代表CML的髓外未成熟原始细胞转化，而非原发性T细胞淋巴瘤。
在排除CML的髓外原始细胞期之前，应怀疑原发性TLBL的诊断。
