Hess J L, Zutter M M, Castleberry R P, Emanuel P D
Lauren V. Ackerman Laboratory of Surgical Pathology, Department of Pathology, Washington University School of Medicine, St. Louis, Missouri 63110, USA.
Am J Clin Pathol. 1996 Feb;105(2):238-48. doi: 10.1093/ajcp/105.2.238.
Juvenile chronic myelogenous leukemia (JCML) is an aggressive myeloproliferative disorder of childhood that differs both clinically and pathologically from adult type, Philadelphia chromosome positive chronic myelogenous leukemia, and from the other myeloproliferative disorders that are more common in adulthood. The disease can have widely varying clinical presentations and shares many features with the monosomy 7 syndrome and chronic myelomonocytic leukemia. With no specific marker chromosome, establishing the diagnosis can be difficult, and relies on a constellation of clinical, pathologic, and laboratory findings. This article discusses the differential diagnosis of JCML with an emphasis on the pathologic findings and laboratory data that are particularly important for confirming the diagnosis. The sensitivity, specificity, and clinical utility of cell culture colony assays are reviewed. Finally, current knowledge of the biology of JCML and some of the controversies regarding this disease are discussed.
青少年慢性粒细胞白血病（JCML）是一种侵袭性的儿童骨髓增殖性疾病，在临床和病理上均不同于成人型费城染色体阳性慢性粒细胞白血病，也不同于其他在成年期更常见的骨髓增殖性疾病。该疾病临床表现差异很大，与7号染色体单体综合征和慢性粒单核细胞白血病有许多共同特征。由于没有特定的标记染色体，确诊可能很困难，需要依靠一系列临床、病理和实验室检查结果。本文讨论JCML的鉴别诊断，重点是对确诊特别重要的病理检查结果和实验室数据。回顾了细胞培养集落测定的敏感性、特异性和临床应用。最后，讨论了JCML的生物学现状以及有关该疾病的一些争议。
