Ohno T, Hada S, Sugiyama T, Mizumoto T, Furukawa H, Nagai K
Department of Internal Medicine, Ohtsu Red Cross Hospital, Shiga, Japan.
Am J Hematol. 1998 Apr;57(4):320-5. doi: 10.1002/(sici)1096-8652(199804)57:4<320::aid-ajh9>3.0.co;2-w.
Although a breakpoint in the minor breakpoint cluster region (m-bcr) of the BCR gene is observed in about two-thirds of patients with Philadelphia chromosome-positive acute lymphoblastic leukemia, this type of genomic rearrangement occurs very rarely in chronic myeloid leukemia (CML). We describe here the eighth case of m-bcr CML, and delineate unique clinical characteristics found in common to the 7 cases reported previously. Monocytosis with a low neutrophil/monocyte ratio resembling chronic myelomonocytic leukemia was the most striking feature of m-bcr CML. Splenomegaly and basophilia were not conspicuous in chronic phase. A high percentage of immature granulocytes and low neutrophil alkaline phosphatase score were the findings in common with classical CML. Lymphoid and myeloid blast changes have been observed at and shortly after presentation so far. We found a hybrid type of blast crisis in the course of m-bcr CML.Thus, m-bcr CML may be a definite subtype of CML, exhibiting distinct clinical characteristics. The presence of fusion product of m-bcr mRNA in an earlier myeloid cell may involve monocytic lineage in addition to myeloproliferative defects.
虽然在约三分之二的费城染色体阳性急性淋巴细胞白血病患者中可观察到BCR基因的微小断点簇区域(m-bcr)出现断点,但这种基因组重排在慢性髓性白血病(CML)中非常罕见。我们在此描述第8例m-bcr CML病例,并阐述先前报道的7例病例共有的独特临床特征。单核细胞增多伴中性粒细胞/单核细胞比率低,类似慢性粒单核细胞白血病,是m-bcr CML最显著的特征。慢性期脾肿大和嗜碱性粒细胞增多不明显。未成熟粒细胞比例高和中性粒细胞碱性磷酸酶评分低是与经典CML共有的表现。到目前为止,在就诊时及就诊后不久已观察到淋巴系和髓系原始细胞变化。我们在m-bcr CML病程中发现了一种混合型原始细胞危象。因此,m-bcr CML可能是CML的一个明确亚型,表现出独特的临床特征。早期髓系细胞中m-bcr mRNA融合产物的存在除了骨髓增殖缺陷外,可能还涉及单核细胞系。