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Leukemia - Acute

Recurrent genetic abnormalities

Acute promyelocytic leukemia with t(V;17)(V;q12)


Reviewer: Syed Zaidi, M.D. (see Reviewers page)
Revised: 17 February 2013, last major update September 2012
Copyright: (c) 2001-2013, PathologyOutlines.com, Inc.

General
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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 #15
● 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))
● 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
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● 23 year old man with tuberculosis (Korean J Hematol 2012;47:229)
● 31 year old woman with t(17;20) masking t(15;17) variant (Cancer Genet Cytogenet 2006;168:73)
● 66 year old man with PRKAR1A gene (Blood 2007;110:4073)

Micro description
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● 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

Micro images
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Cells with many granules, regular nuclei, no Auer rods


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

Molecular / cytogenetics description
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● 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) variantw

Differential diagnosis
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● t(11;17) may resemble AML with 11q23 abnormality (Cancer Genet Cytogenet 2005;159:168)

End of Leukemia - Acute > Recurrent genetic abnormalities > Acute promyelocytic leukemia with t(V;17)(V;q12)


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