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High hyperdiploid acute lymphoblastic leukaemia

Written1999-06Barbara Gibbons
NE London Regional Cytogenetics Laboratory 2nd Floor, Queen Square House Institute of Neurology Queen Square London WC1N 3BG, UK

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Identity

ICD-Topo C420,C421,C424 BLOOD, BONE MARROW, & HEMATOPOIETIC SYS
ICD-Morpho 9811/3 B lymphoblastic leukaemia/lymphoma, NOS
ICD-Morpho 9815/3 B lymphoblastic leukaemia/lymphoma with hyperdiploidy
Atlas_Id 1085

Clinics and Pathology

Disease acute lymphoblastic leukaemia (ALL)
Phenotype / cell stem origin c-ALL or early pre-B immunophenotype
Epidemiology this type of leukaemia is most common in children (20-30% of childhood ALL), peaking at 3-5 years although it is rare in infants and has a low incidence in adults (5% of adult ALL); excess of females
Clinics high hyperdiploidy is associated with a low white cell count, and FAB type L1 or L2
Prognosis prognosis for children is good with long term survival of 70%-80%; adults do less well although event free survival of 59% at 3 years has been reported; the good prognosis is thought to be related to leukaemic cell sensitivity to a number of anti-leukaemic drugs and the propensity of cells to respond by apoptosis; it is unclear why a proportion of patients fail to achieve long term remission but there is evidence that higher chromosome counts of >56 chromosomes and the presence of trisomies 4 and 10 may be associated with a good prognosis whereas the presence of i(17q) is clearly associated with a poor prognosis

Cytogenetics

Note the hyperdiploid karyotype is thought to arise from a from a single step mechanism; maintenance of heterozygosity has been demonstrated suggesting that the hyperdiploidy does not arise from a near haploid precursor
Cytogenetics Morphological patients with hyperdiploidy of >50 chromosomes have clones of 51-68 chromosomes; although high hyperdiploid clones are rarely identical, they tend to show a pattern of chromosome gain with extra copies of chromosomes 4, 6, 10, 14, 18 and 21; the gains, apart from chromosome 21, more often result in trisomy rather than tetrasomy for the gained chromosomes
Cytogenetics Molecular high hyperdiploidy can be detected by application of a panel of probes which will detect the characteristic pattern of gain; this technique can be applied to interphase blasts but care must be exercised in interpretation and the non-random translocations such as t(9;22), t(1;19), t(12;21) and t(4;11) should be excluded by application of appropriate probes
Additional anomalies translocations and other structural chromosome abnormalities are present in approximately half of high hyperdiploid cases; duplication 1q is the most common additional change; duplication of 1q, deletion of 6q and random structural abnormalities have no known prognostic impact; however, the presence of non-random translocations such as t(9;22), t(4;11), t(1;19) and t(12;21) indicate that the translocation is most likely the primary change and that the hyperdiploidy is probably a secondary event; in such cases the leukaemia should be classified according to the translocation rather than the ploidy group in order to assign the correct prognostic implications

Bibliography

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Hyperdiploid acute lymphoblastic leukemia with 51 to 65 chromosomes: a distinct biological entity with a marked propensity to undergo apoptosis.
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Favorable prognosis of hyperdiploid common acute lymphoblastic leukemia may be explained by sensitivity to antimetabolites and other drugs: results of an in vitro study.
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PMID 7833478
 
Dichotomy of hyperdiploid acute lymphoblastic leukemia on the basis of the distribution of gained chromosomes.
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Formation of a hyperdiploid karyotype in childhood acute lymphoblastic leukemia.
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PMID 1351763
 
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Heterogeneity of hyperdiploid (51-67) childhood acute lymphoblastic leukemia.
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Detection of hyperdiploid karyotypes (>50 chromosomes) in childhood acute lymphoblastic leukemia (ALL) using fluorescence in situ hybridization (FISH).
Ritterbach J, Hiddemann W, Beck JD, Schrappe M, Janka-Schaub G, Ludwig WD, Harbott J, Lampert F
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Cytogenetics adds independent prognostic information in adults with acute lymphoblastic leukaemia on MRC trial UKALL XA. MRC Adult Leukaemia Working Party.
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Citation

This paper should be referenced as such :
Gibbons, B
High hyperdiploid acute lymphoblastic leukaemia
Atlas Genet Cytogenet Oncol Haematol. 1999;3(3):145-146.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/hyperploidID1085.html


External links

COSMICHisto = - Site = haematopoietic_and_lymphoid_tissue (COSMIC)
arrayMapTopo ( C42) Morph ( 9811/3) - arrayMap (UZH-SIB Zurich)  [auto + random 100 samples .. if exist ]   [tabulated segments]
arrayMapTopo ( C42) Morph ( 9815/3) - arrayMap (UZH-SIB Zurich)  [auto + random 100 samples .. if exist ]   [tabulated segments]
 
 
Disease databaseHigh hyperdiploid acute lymphoblastic leukaemia
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