Last update:
12-Jun-2001

Arch Hellen Med, 17(6), November-December 2000, 576-583

REVIEW

Leukemogenesis in childhood
Etiologic factors and pathogenetic mechanisms

J.P. PANAGIOTOU, S. POLYCHRONOPOULOU-ANDROULAKAKI
^{Department of Pediatric Hematology-Oncology, “Aghia Sophia” Children’s Hospital, Athens, Greece}

Acute leukemia in children accounts for approximately one-third of pediatric malignancies and is the commonest among them. Leukemia represents an excellent example of successful scientific research and therapeutic achievment in cancer, although there remain many unanswered questions concerning its etiology at the cellular and molecular level. Although several environmental and genetic factors, such as ionizing radiation, toxic chemicals, antineoplastic drugs and certain genetic diseases, have been linked with an increased risk of developing leukemia, the etiologic basis of the disease, especially in children, remains unclear. There is strong evidence for considering acute leukemia in children as a genetic disease, in that somatically acquired genetic changes may play a major role in its pathogenesis. However, no specific predisposing genetic condition directly resulting in leukemic transformation of normal lymphocytic and myelocytic progenitor cells has been yet recognized. In some cases, these somatic changes are superimposed on wellknown inherited-gene alterations, such as deletions of the neurofibromatosis type I gene (NF-1 gene). In this review article, the factors linked with increased leukemic transformation and the possible pathogenetic mechanisms through which these factors may act at the molecular or cellular level are discussed.

Key words: Acute leukemia, Childhood, Leukemogenesis, Predisposing factors.