Objective: The purpose of this study was to determine the clinical and biological characteristics at diagnosis in children and adolescents with chronic myelogenous leukemia (CML) in contemporary practice.
Methods: Analysis was conducted on data from 3 prospective trials conducted in children and adolescents with CML. Forty pediatric patients were evaluated in 16 French pediatric oncology units between 1991 and 2003.
Results: The disease predominately affected children who were older than 10 years (67% of the patients), with a higher prevalence in boys than girls (gender ratio: 1.5). Approximately 20% of cases were diagnosed incidentally. The main presenting symptoms were asthenia, weight loss, and complaints related to splenomegaly. Occasional patients presented with signs evocative of leukostasis. Symptoms were more common in patients with splenic enlargement, which was present in 70% of patients, and higher leukocyte counts. Markedly raised leukocyte counts were common (median white blood cell count: 242 x 10(9)/L). The age and the gender of the patients had no effect on the leukocyte count, the hemoglobin level, or the platelet count. A predominance of b3a2 transcript was observed in the 16 children who were studied for the type of chimeric BCR-ABL mRNA.
Conclusions: This is largest reported series of CML at diagnosis in children and adolescents. It shows that the characteristics of CML seem to differ in children compared with previously published adult series; in particular, the presenting leukocyte counts are often higher in children.