Akashi K, Mizuno S, Harada M, Kimura N, Kinjyo M, Shibuya T, Shimoda K, Takeshita M, Okamura S, Matsumoto I
Department of Hematology, Harasanshin General Hospital, Fukuoka, Japan.
Exp Hematol. 1993 Jun;21(6):743-8.
We describe 2 cases of "bilineal" crisis in chronic myelogenous leukemia (CML) with T cell and myeloid phenotypes. In both cases, morphocytochemically distinct myeloid and T lymphoid blast populations proliferated simultaneously in the phase of blastic crisis--myeloperoxidase (MPO)-positive, CD7+/CD33+ myeloblasts in the peripheral blood, and MPO-negative, periodic acid Schiff (PAS)-positive lymphoblasts in the lymph nodes. In each case, common karyotypes containing Ph1 translocation were demonstrated in both the peripheral blood and the lymph node samples. In Case 1, the lymph nodes were occupied by > 90% lymphoblasts, which were positive for CD2, cytoplasmic CD3 (cCD3), CD5 and CD7 and terminal deoxynucleotidyl transferase (TdT), but negative for myeloid antigens. Myeloblasts and T lymphoblasts showed an identical rearrangement of the bcr gene by Southern blotting analysis, although the clonal rearrangement of the T cell receptor (TcR)-delta gene was seen only in T lymphoblasts. In Case 2, simultaneous proliferation of myeloblasts and lymphoblasts was documented morphocytochemically in the lymph node, and a flow cytometric analysis revealed the coexistence of CD7+/CD33+ and CD7+/CD33- blast populations. Each blast population was enriched by antibody-conjugated immunomagnetic beads; the former was positive for MPO by 64% but negative for cCD3 and TdT, whereas the latter was positive for cCD3 and TdT but negative for MPO (< 1%). CD7+/CD33+ myeloblasts and CD7+/CD33- lymphoblasts showed an identical rearrangement of the bcr gene. Neither TcR-beta, TcR-gamma nor the TcR-delta gene was clonally rearranged in either population. These observations clearly indicate that T lymphoid and myeloid blasts share common Ph1-positive progenitors, and that Ph1-positive T lymphoid/myeloid progenitors are probably involved in the development of blastic transformation in some percentage of CML patients.
我们描述了2例慢性粒细胞白血病(CML)出现具有T细胞和髓系表型的“双系”危象的病例。在这两例中,形态细胞化学上不同的髓系和T淋巴母细胞群体在急变期同时增殖——外周血中髓过氧化物酶(MPO)阳性、CD7+/CD33+的髓母细胞,以及淋巴结中MPO阴性、过碘酸希夫(PAS)阳性的淋巴母细胞。在每例中,外周血和淋巴结样本均显示含有Ph1易位的常见核型。病例1中,淋巴结被>90%的淋巴母细胞占据,这些细胞CD2、胞质CD3(cCD3)、CD5和CD7以及末端脱氧核苷酸转移酶(TdT)呈阳性,但髓系抗原呈阴性。通过Southern印迹分析,髓母细胞和T淋巴母细胞显示bcr基因的重排相同,尽管T细胞受体(TcR)-δ基因的克隆重排仅在T淋巴母细胞中可见。病例2中,淋巴结经形态细胞化学证实髓母细胞和淋巴母细胞同时增殖,流式细胞术分析显示CD7+/CD33+和CD7+/CD33-母细胞群体共存。每个母细胞群体通过抗体偶联免疫磁珠富集;前者64%的细胞MPO呈阳性,但cCD3和TdT呈阴性,而后者cCD3和TdT呈阳性,但MPO呈阴性(<1%)。CD7+/CD33+髓母细胞和CD7+/CD33-淋巴母细胞显示bcr基因的重排相同。在任一群体中,TcR-β、TcR-γ和TcR-δ基因均未发生克隆重排。这些观察结果清楚地表明,T淋巴母细胞和髓母细胞共享常见的Ph1阳性祖细胞,并且Ph1阳性T淋巴/髓系祖细胞可能在一定比例的CML患者急变转化的发生中起作用。