Congenital Syndromes and Leukemia: Clues to Pathogenesis

Shai Izraeli*

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review


The association of specific congenital syndromes with leukemia provides an opportunity to study the process of leukemogenesis on the background of known genetic alterations. The role of the intracellular DNA damage response system in suppressing leukemia is demonstrated by the congenital disorders of genomic instability. Specific collaborations between survival and differentiation pathways characterize the leukemias observed in Down, Noonan and neurofibromatosis syndromes. As these syndromes clearly reveal, childhood leukemia arises when the delicate balance between growth, development and differentiation of the fetal and early post-natal hematopoietic system is disrupted.

Original languageEnglish
Pages (from-to)246-260
Number of pages15
JournalReviews in Clinical and Experimental Hematology
Issue number3
StatePublished - Sep 2003


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