Iwamasa K, Yasukawa M, Fujita S
First Department of Internal Medicine, School of Medicine, Ehime University, Japan.
Jpn J Med. 1989 Jan-Feb;28(1):89-95. doi: 10.2169/internalmedicine1962.28.89.
We report a case of blast crisis in chronic myelogenous leukemia (CML) in which T lymphocytic and megakaryocytic lineages were both involved. A 55-year-old male was initially admitted to Ehime University Hospital because of generalized lymphadenopathy. The morphological features of peripheral blood and bone marrow were consistent with chronic phase of CML. Cytogenetic studies of bone marrow and lymph node cells both showed the Ph1 chromosome with additional abnormalities. The patient was diagnosed as being in the extramedullary blast crisis of CML involving lymph nodes. After six months, blasts increased in bone marrow and peripheral blood. The phenotypes of lymph node blasts were positive for CD2, CD7 and TdT, but negative for CDw41 (platelet glycoprotein IIb/IIIa). On the other hand, those of peripheral blood blasts were positive for CDw41, but negative for CD2, CD7 and TdT. Chromosome studies of lymph node cells and bone marrow cells revealed 46, XY, inv(7) (p15q34), t(9;22) (q34;q11) and 46, XY, t(1;3) (q23:q21), t(9;22) (q34;q11), respectively. The rearrangement of T cell receptor beta chain gene was detected in lymph node blasts, but not in peripheral blood blasts. The identity of the rearrangement patterns of the breakpoint cluster region on chromosome 22 was detected in these blasts. According to these data, it was suggested that blast crisis of CML occurred in two distinct lineages, T lymphocyte and megakaryocyte.
我们报告了一例慢性粒细胞白血病（CML）急变期病例，其中T淋巴细胞系和巨核细胞系均受累。一名55岁男性因全身淋巴结肿大最初入住爱媛大学医院。外周血和骨髓的形态学特征与CML慢性期相符。骨髓和淋巴结细胞的细胞遗传学研究均显示有Ph1染色体及其他异常。该患者被诊断为CML髓外急变期累及淋巴结。6个月后，骨髓和外周血中的原始细胞增多。淋巴结原始细胞的表型CD2、CD7和TdT呈阳性，但CDw41（血小板糖蛋白IIb/IIIa）呈阴性。另一方面，外周血原始细胞的表型CDw41呈阳性，但CD2、CD7和TdT呈阴性。淋巴结细胞和骨髓细胞的染色体研究分别显示为46, XY, inv(7) (p15q34), t(9;22) (q34;q11) 和46, XY, t(1;3) (q23:q21), t(9;22) (q34;q11)。在淋巴结原始细胞中检测到T细胞受体β链基因重排，但在外周血原始细胞中未检测到。在这些原始细胞中检测到22号染色体上断裂簇区域重排模式的一致性。根据这些数据，提示CML急变期发生在两个不同的细胞系，即T淋巴细胞系和巨核细胞系。
