Carlsen N L, Erichsen G, Lisse I
Department of Paediatrics, State University Hospital, Rigshospitalet, Copenhagen, Denmark.
Anticancer Res. 1990 Nov-Dec;10(6):1739-41.
A three-and-a-half-year-old boy presented in the blastic phase of chronic myelocytic leukaemia (CML) with lymphoblastic infiltration of CNS and testes. The clinical signs and symptoms and also blood and bone marrow findings were otherwise compatible with the chronic phase of the disease, and none of the factors predictive of early transformation were present. Cytogenetic analysis revealed that the Ph1 chromosome, with no additional chromosomal abnormalities, was present in 85% of the bone marrow cells. Meningeal leukaemia is almost unknown in the chronic phase of CML. However, the incidence in the blastic phase may resemble the incidence of CNS leukaemia at diagnosis in children with acute leukaemias. Testicular involvement appears to be extremely rare even in the blastic phase of CML; the "true" incidence may, however, also resemble that of acute leukaemias. This raises the question of the need for testicular and meningeal surveillance and prophylaxis, at least during the blastic phase of CML. One should consider whether the simultaneous meningeal and testicular lymphoblastic leukaemia in this patient was the result of blastic transformation at two independent sites, or whether the testes were seeded from the meninges without identifiable spread to blood and bone marrow.
一名3岁半男孩以慢性粒细胞白血病（CML）急变期就诊，伴有中枢神经系统和睾丸的淋巴细胞浸润。临床症状体征以及血液和骨髓检查结果在其他方面与疾病慢性期相符，且不存在任何预测早期转变的因素。细胞遗传学分析显示，85%的骨髓细胞中存在Ph1染色体，无其他染色体异常。脑膜白血病在CML慢性期几乎不为人知。然而，急变期的发病率可能与急性白血病患儿诊断时中枢神经系统白血病的发病率相似。即使在CML急变期，睾丸受累似乎也极为罕见；不过，“实际”发病率可能也与急性白血病相似。这就引发了一个问题，即至少在CML急变期是否需要对睾丸和脑膜进行监测及预防。人们应该考虑该患者同时出现的脑膜和睾丸淋巴细胞白血病是两个独立部位急变的结果，还是睾丸是由脑膜播散而来，而未发现有向血液和骨髓的扩散。
