Chapter

Acute Promyelocytic Leukemia

Volume 313 of the series Current Topics in Microbiology and Immunology pp 85-100

The Theory of APL Revisited

  • P. P. ScaglioniAffiliated withCancer Biology and Genetics Program, Memorial Sloan-Kettering Cancer Center, Sloan-Kettering Institute
  • , P. P. PandolfiAffiliated withCancer Biology and Genetics Program, Memorial Sloan-Kettering Cancer Center, Sloan-Kettering Institute

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Abstract

Acute promyelocytic leukemia (APL) is associated with reciprocal and balanced chromosomal translocations always involving the retinoic acid receptor α (RARα) gene on chromosome 17 and variable partner genes (X genes) on distinct chromosomes. RARα fuses to the PML gene in the majority of APL cases, and in a few cases to the PLZF, NPM, NuMA and STAT5b genes. As a consequence, X-RARα and RARα-X fusion genes are generated encoding aberrant chimeric proteins that exert critical oncogenic functions. Here we will integrate some of the most recent findings in APL research in a unified model and discuss some of the outstanding questions that remain to be addressed.