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Acute Promyelocytic Leukemia (APL)

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Encyclopedic Reference of Cancer

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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 the myelopoiesis. According to the French-American-British (FAB) classification of acute leukemia, APL corresponds to the M3 and M3-Variant subtypes, and accounts for 10–15% of adult AML patients. APL is distinct from other subtypes of leukemia because of its invariable association with reciprocal chromosomal translocations involving the retinoic acid receptor α (RARα) gene on chromosome 17 (Table) and by its sensitivity to the differentiating action of all-trans retinoic acid (ATRA).

Table 1 Acute Promyelocytic Leukemia (APL). Table - Molecular Genetics of Acute Promyelocytic Leukemia.
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References

  1. Warrell RP Jr, de The H, Wang ZY, Degos L (1993) Acute promyelocytic leukemia. New England Journal of Medicine 329:177–189

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  2. He LZ, Guidez F, Tribioli C, Peruzzi D, Ruthardt M, Zelent A, Pandolfi PP (1998) Distinct interactions of PML-RARα and PLZF-RARα with co-repressors determine differential responses to RA in APL. Nat Genet 18:126–135

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  3. He LZ, Merghoub T, Pandolfi PP (1999) In vivo analysis of the molecular pathogenesis of Acute Promyelocytic Leukemia in the mouse and its therapeutic implications. Oncogene 18:5278–5292

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  4. Rego EM, He LZ, Warrell RP, Jr., Wang ZG, Pandolfi PP (2000) Retinoic acid (RA) and As2O3 treatment in transgenic models of acute promyelocytic leukemia (APL) unravel the distinct nature of the leukemogenic process induced by the PML-RARα and PLZF-RARα oncoproteins. Proc Natl Acad Sci of USA 97:10173–10178

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Authors and Affiliations

  1. Memorial Sloan-Kettering Cancer Center, Cornell University, New York, NY, USA

    Li-Zhen He, Eduardo M. Rego & Pier Paolo Pandolfi

Authors
  1. Li-Zhen He
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  2. Eduardo M. Rego
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  3. Pier Paolo Pandolfi
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Editor information

Editors and Affiliations

  1. Division Cytogenetics - H-0400, German Cancer Research Center, Im Neuenheimer Feld, 69120, Heidelberg, Germany

    Manfred Schwab (Professor for Genetics, Director) (Professor for Genetics, Director)

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© 2001 Springer-Verlag

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He, LZ., Rego, E.M., Pandolfi, P.P. (2001). Acute Promyelocytic Leukemia (APL). In: Schwab, M. (eds) Encyclopedic Reference of Cancer. Springer, Berlin, Heidelberg. https://doi.org/10.1007/3-540-30683-8_18

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  • DOI: https://doi.org/10.1007/3-540-30683-8_18

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-540-66527-4

  • Online ISBN: 978-3-540-30683-2

  • eBook Packages: Springer Book Archive

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