Leukemia acute
Recurrent genetic abnormalities
Acute promyelocytic leukemia with t(V;17)(V;q12)

Topic Completed: 1 February 2013

Revised: 19 November 2019

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PubMed Search: Acute promyelocytic leukemia [title] variant [title]

Syed Zaidi, M.D.
Page views in 2019: 343
Page views in 2020 to date: 94
Cite this page: Zaidi S. Acute promyelocytic leukemia with t(V;17)(V;q12). PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/leukemiatv17.html. Accessed April 1st, 2020.
Definition / general
  • Note: "variant" APL without further description may mean microgranular (morphologic) variant or cytogenetic variant other than t(15;17)
  • Uncommon, involves retinoic acid receptor alpha on #17 but not PML gene on #1
  • t(11;17) is most common; also called ZBTB16-RARα variant
  • Symptoms: disseminated intravascular coagulation / DIC common (Atlas of Genetics and Cytogenetics: t(11;17)(q23;q21) ZBTB16/RARA [Accessed 2 April 2018])
  • May NOT respond to all-trans retinoic acid therapy; may be more aggressive than classic APL (Blood 1995;85:1083)
  • Recommended to combine cytogenetics, FISH and molecular biology to document presence / absence of PML-RARα fusion gene in complex cases (Cancer Genet Cytogenet 2005;159:69)
Case reports
Microscopic (histologic) description
  • Features are intermediate between hypergranular acute promyelocytic leukemia (M3) and acute leukemia with maturation (M2), most cells have many granules and regular nuclei; usually no Auer rods but increased pseudo Pelger-Huet cells
Microscopic (histologic) images

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Cells with many granules, regular nuclei, no Auer rods

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t(11;17)(q23;q21) variant shows promyelocytes in peripheral blood and marrow

Molecular / cytogenetics description
  • Involves RAR alpha and either PLZF / ZBTB16 (11q23), NUMA (11q13), NPM (5q31) or STAT5b genes (Leukemia 2002;16:1927)
Molecular / cytogenetics images

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t(11;17)(q23;q21) variant

Differential diagnosis
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