Hasle H, Heim S, Schroeder H, Schmiegelow K, Ostergaard E, Kerndrup G
Department of Pediatrics, Odense University Hospital, Denmark.
Leukemia. 1995 Apr;9(4):605-8.
Pancytopenia followed by a period of spontaneous recovery may precede the diagnosis of acute lymphoblastic leukemia (pre-ALL). Although both pre-ALL and myelodysplastic syndromes are preleukemic in a strictly temporal sense, there are several marked differences between the two conditions. We present eight children with pre-ALL who represented 2% of all cases of childhood ALL. The bone marrow was normo- or hypocellular with increased reticulin fibrosis during the pre-ALL phase. No cytogenetic abnormalities were found at the pre-ALL phase, but had developed at the time of overt leukemia in four of the six children examined. Based on the findings in our patients and on cases reported in the literature, we argue that pre-ALL is likely to represent a paraneoplastic syndrome early in the leukemic development that might be mediated via inhibitory properties related to clonally expanding but still cytogenetically normal cells. The findings may indicate a multistep pathogenesis of ALL.
全血细胞减少随后经历一段自发恢复时期，可能先于急性淋巴细胞白血病（白血病前期）的诊断出现。虽然从严格的时间意义上来说，白血病前期和骨髓增生异常综合征都属于白血病前期疾病，但这两种情况之间存在一些显著差异。我们报告了8例白血病前期的儿童患者，占儿童急性淋巴细胞白血病所有病例的2%。在白血病前期阶段，骨髓细胞数量正常或减少，网硬蛋白纤维化增加。在白血病前期阶段未发现细胞遗传学异常，但在接受检查的6名儿童中，有4名在白血病明显发作时出现了细胞遗传学异常。基于我们患者的研究结果以及文献报道的病例，我们认为白血病前期可能代表白血病发展早期的一种副肿瘤综合征，可能是由与克隆性扩增但细胞遗传学仍正常的细胞相关的抑制特性介导的。这些发现可能表明急性淋巴细胞白血病存在多步骤发病机制。
