Leukemia - Acute
B lymphoblastic leukemia / lymphoma with hyperdiploidy
Reviewer: Daniela Mihova, M.D. (see Reviewers page)
Revised: 1 February 2013, last major update September 2012
Copyright: (c) 2001-2013, PathologyOutlines.com, Inc.
● “High hyperdiploidy” means >50 chromosomes and <66 chromosomes without translocations or other structural alternations
● Generally arises by simultaneous gain of all additional chromosomes in a single abnormal mitosis (Genes Chromosomes Cancer 2005;44:113)
● Hyperdiploidy appears to be an early event occurring prenatally (Genes Chromosomes Cancer 2004;40:38, Leukemia 2003;17:2202, Blood 2002;100:347)
● Affects 25-33% of children (usually age 3-5 years) and 5% of adults with B ALL; not seen in infants
● Favorable overall survival (Am J Hematol 2008;83:34), although outcome in children varies by specific trisomy present (Blood 2003;102:2756)
● Immunophenotype: CD19+, CD10-, CD34+ and CD45-
● No distinct morphology
● Mutations in NRAS (10%), FLT3 (9%), PTPN11 (9%) and KRAS (6%, Genes Chromosomes Cancer 2008;47:26)
● Most common extra copies are 21, X, 14 and 4; least often 1, 2, 3
● Best prognosis of simultaneous trisomies: 4, 10 and 17
End of Leukemia - Acute > PreB ALL > B lymphoblastic leukemia / lymphoma with hyperdiploidy
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