JUVENILE MYELOMONOCYTIC LEUKEMIA - MOLECULAR UNDERSTANDING AND PROSPECTS FOR THERAPY

Juvenile myelomonocytic leukemia is a rare but deadly myeloproliferati ve disorder of early childhood that infrequently progresses to acute l eukemia. The pathogenesis of this leukemia has been linked to deregula ted signal transduction, resulting in growth factor hypersensitivity. Several other myeloproliferative disorders appear to share growth fact or hypersensitivity as a common pathophysiological mechanism and thus this leukemia serves as an important model. New treatment modalities, such as retinoid therapy, are emerging for juvenile myelomonocytic leu kemia. Further understanding of deregulated signal transduction should pave the way for even more rationally designed therapy for this leuke mia and related disorders.