Encyclopedia of Cancer

Living Edition
| Editors: Manfred Schwab

Acute Promyelocytic Leukemia

  • Li-Zhen He
  • Lorena L. Figueiredo-Pontes
  • Eduardo M. Rego
  • Pier Paolo Pandolfi
Living reference work entry
DOI: https://doi.org/10.1007/978-3-642-27841-9_63-2

Definition

Acute promyelocytic leukemia (APL) is a distinct subtype of acute myeloid leukemia (AML) characterized by the expansion of leukemic cells blocked at the promyelocytic stage of myelopoiesis. According to the French–American–British (FAB) classification of acute leukemia, APL corresponds to the M3 and M3-variant subtypes, and according to World Health Organization classification (2001) it corresponds to the subtype: AML associated with translocations involving chromosomes 15 and 17 [t(15;17)] and variants. APL accounts for 5–10 % of adult AML patients in Caucasian populations and for 20–30 % among patients with Latino ancestry. Invariably, APL leukemic cells harbor chromosomal translocations involving the retinoic acid receptor α ( RARα) gene on chromosome 17 (Table 1), which may be fused to one of five possible partner genes: promyelocytic leukemia ( PML), promyelocytic leukemia zinc finger ( PLZF), nucleophosmin ( NPM), nuclear mitotic apparatus ( NuMA), and signal transducer and...

Keywords

Acute Myeloid Leukemia Retinoic Acid Disseminate Intravascular Coagulation Acute Promyelocytic Leukemia Leukemic Blast 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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References

  1. Lin H-K, Bergmann S, Pandolfi PP (2005) Deregulated TGF-β signaling in leukemogenesis. Oncogene 24:5693–5700CrossRefPubMedGoogle Scholar
  2. Rego EM, Pandolfi PP (2002) Reciprocal products of chromosomal translocations in human cancer pathogenesis: key players or innocent bystanders? Trends Mol Med 8:396–405CrossRefPubMedGoogle Scholar
  3. Rego EM, Ruggero D, Tribioli C et al (2006) Leukemia with distinct phenotypes in transgenic mice expressing PML/RAR alpha, PLZF/RAR alpha or NPM/RAR alpha. Oncogene 25(13):1974–1979CrossRefPubMedGoogle Scholar
  4. Sanz M (2006) Treatment of acute promyelocytic leukemia. Hematol/Am Soc Hematol Educ Program 147–155Google Scholar
  5. Scaglioni PP, Pandolfi PP (2007) The theory of acute promyelocytic leukemia revisited. Curr Top Microbiol Immunol 313:85–100PubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  • Li-Zhen He
    • 1
  • Lorena L. Figueiredo-Pontes
    • 2
  • Eduardo M. Rego
    • 2
  • Pier Paolo Pandolfi
    • 3
  1. 1.Memorial Sloan-Kettering Cancer CenterWeill Cornell Graduate School of Medical SciencesNew YorkUSA
  2. 2.Medical School of Ribeirão PretoUniversity of São PauloRibeirão PretoBrazil
  3. 3.Division of GeneticsBeth Israel Deaconess Medical CenterBostonUSA