Leone G, La Rocca L M, Teofili L, De Candia E, Landolfi R, Sica S, Zini G, Zollino M, Tabilio A
Istituti di Semeiotica Medica, Università Cattolica del S. Cuore, Roma, Italy.
Haematologica. 1992 Jul-Aug;77(4):311-4.
Chronic myeloid leukemia arises from a somatic mutation in a pluripotent stem cell. It generally terminates with a blastic crisis (BC). One third of BC are lymphoid, and most have a pre-B phenotype. Few cases of T-lymphoid BC have been reported. Here we describe a lymph node blast crisis mimicking T-immunoblastic lymphoma.
Bone marrow and lymph nodes were histologically examined by standard methods and by an immunoperoxidase technique. Cytogenetic studies were also performed on lymph node and blood cells. Analysis of T-cell receptor genes and BCR rearrangements were performed on DNA extracted from both frozen bone marrow and lymph-node cells.
Lymph-node histology showed an infiltration by large lymphoid blasts, consistent with a diagnosis of immunoblastic lymphoma. Blast cells were CD2, CD7, TDT positive, and negative for myeloid and mature lymphoid antigens. The Ph1 chromosome was found in both bone marrow and lymph-node cells. BCR rearrangement was found in the DNA from both bone marrow and lymph-node cells. TCR genes were not rearranged.
The present study provides strong evidence that the lymph-node blast crisis of CML can assume the morphological appearance of immunoblastic lymphoma and may retain the immunological phenotype and genetic features of early T cells with BCR rearrangements.
慢性髓性白血病起源于多能干细胞的体细胞突变。它通常以急变期(BC)告终。三分之一的急变期为淋巴细胞性,且大多数具有前B表型。T淋巴细胞性急变期的病例报道较少。在此我们描述一例酷似T免疫母细胞淋巴瘤的淋巴结急变期。
采用标准方法及免疫过氧化物酶技术对骨髓和淋巴结进行组织学检查。还对淋巴结和血细胞进行了细胞遗传学研究。对从冷冻骨髓和淋巴结细胞中提取的DNA进行T细胞受体基因和BCR重排分析。
淋巴结组织学显示有大量淋巴母细胞浸润,符合免疫母细胞淋巴瘤的诊断。母细胞CD2、CD7、末端脱氧核苷酸转移酶(TDT阳性,髓系和成熟淋巴细胞抗原阴性。在骨髓和淋巴结细胞中均发现了Ph1染色体。在骨髓和淋巴结细胞的DNA中均发现了BCR重排。TCR基因未重排。
本研究提供了有力证据,表明慢性髓性白血病的淋巴结急变期可呈现免疫母细胞淋巴瘤的形态外观,并可能保留具有BCR重排的早期T细胞的免疫表型和遗传特征。