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慢性髓系白血病的血液学分类

Haematological classification of the chronic myeloid leukaemias.

作者信息

Shepherd P C, Ganesan T S, Galton D A

出版信息

Baillieres Clin Haematol. 1987 Dec;1(4):887-906. doi: 10.1016/s0950-3536(87)80031-8.

Abstract

Chronic myeloid leukaemia (CML) includes five subtypes, and the term should be used in the same way as the term chronic lymphoid leukaemia to refer to a group of related conditions. The subtypes of CML are: 1. Chronic granulocytic leukaemia (CGL) (95% of all CML; 90% are Ph+, BCR+, 5% are Ph-, BCR+); 2. Juvenile CML (extremely rare; Ph-, BCR- in the few so far examined); 3. Chronic neutrophilic leukaemia (CNL) (extremely rare; Ph-, BCR- in the few so far examined); 4. Chronic myelomonocytic leukaemia (CMML). CMML with low or normal leukocyte counts is classified as a myelodysplastic syndrome; CMML with high leukocyte count is both myelodysplastic and myeloproliferative. Ph-, BCR-; 5. Atypical CML (aCML). Intermediate between CGL and CMML but has distinctive features. Ph-, mostly BCR-. Significance of few reported BCR+ uncertain. Markedly worse survival than CGL and probably worse than CMML. Definition needs refining. Types 2, 3, 4 and 5 account for 5% of all CML. CGL, CMML, aCML and CNL can be diagnosed in the great majority of cases from the morphological profile of presentation peripheral blood films, but high-quality Romanowsky staining is essential.

摘要

慢性髓系白血病(CML)包括五个亚型,该术语的使用方式应与慢性淋巴细胞白血病相同,用于指代一组相关病症。CML的亚型如下:1. 慢性粒细胞白血病(CGL)(占所有CML的95%;90%为Ph阳性、BCR阳性,5%为Ph阴性、BCR阳性);2. 青少年CML(极为罕见;在目前检查的少数病例中为Ph阴性、BCR阴性);3. 慢性中性粒细胞白血病(CNL)(极为罕见;在目前检查的少数病例中为Ph阴性、BCR阴性);4. 慢性粒单核细胞白血病(CMML)。白细胞计数低或正常的CMML归类为骨髓增生异常综合征;白细胞计数高的CMML兼具骨髓增生异常和骨髓增殖特征。Ph阴性、BCR阴性;5. 非典型CML(aCML)。介于CGL和CMML之间,但有独特特征。Ph阴性,大多为BCR阴性。少数报告为BCR阳性的意义尚不确定。生存期明显短于CGL,可能也短于CMML。定义需要完善。2、3、4和5型占所有CML的5%。在绝大多数病例中,CGL、CMML、aCML和CNL可根据就诊时外周血涂片的形态学特征做出诊断,但高质量的罗曼诺夫斯基染色至关重要。

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