9p Rearrangements in ALL
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| Written | 1999-08 | Nyla A Heerema |
| The Ohio State University, Division of Clinical Pathology, Department of Pathology, 167 Hamilton Hall, 1645 Neil Ave, Columbus, OH 43210, USA |
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1. Identity
| ICD-Topo | C420,C421,C424 BLOOD, BONE MARROW, & HEMATOPOIETIC SYS |
| ICD-Morpho | 9811/3 B lymphoblastic leukaemia/lymphoma, NOS |
| ICD-Morpho | 9837/3 T lymphoblastic leukaemia/lymphoma |
| Atlas_Id | 1156 |
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| del(9p) G- banding - Courtesy Jean-Luc Lai and Alain Vanderhaegen (left), and, from middle left to right: del(9)(p13), del(9)(p21), and del(9)(p22) - Courtesy Diane H. Norback, Eric B. Johnson, and Sara Morrison-Delap, UW Cytogenetic Services; Hybridization with Vysis CDKN2A/CEP9 probe showing 2 green and 2 red signals on normal and one red signal on metaphase with 9p deletion. – Courtesy Adriana Zamecnikova. | |
2. Clinics and Pathology
| Disease | acute lymphocycic leukemia (ALL) |
| Phenotype / cell stem origin | lack of specificity for a particular immunophenotype |
| Epidemiology | approximately 10% of childhood ALL, similar in adult ALL |
| Prognosis | recent data indicate that an abnormal 9p is an adverse risk factor for B-lineage, but not T-lineage pediatric patients; this is more pronounced in standard-risk patients (age 1 - 9 years with WBC count 9/l); a dic(9;12)(p11-13;p11-12) has been reported to have a very low relapse rate |
3. Cytogenetics
| Cytogenetics Morphological | various aberrations result in an abnormal 9p; these include monosomy 9, del(9p), add(9p), der(9)t(V;9)(V;p), dic(V;9)(V;p), i(9q) and balanced translocations with 9p breakpoints; dicentric chromosomes in ALL nearly always involve a chromosome 9; an abnormal 9p usually occurs as part of a more complex karyotype; it occurs as a sole aberration in less than 10% of cases with an abnormal 9p |
| Additional anomalies | additional anomalies are frequent; an abnormal 12p is particularly frequent (16% of cases), and a deletion of 6q is also frequent (11% of cases) |
4. Genes involved and Proteins
| Note | the different types of 9p aberrations may have different molecular consequences; when a deletion of 9p occurs, the genes involved colud be MTS1/CDK4I/p16INK4A(CDKN2) and MTS2/p15INK4B(CDKN2B); these are believed to be tumor suppressor genes, and loss of heterozygosity occurs more frequently than cytogenetic deletions of 9p; however, mutation of the remaining allele is infrequent, and methylation changes may cause inactivation of the remaining allele |
5. Bibliography
| Dicentric (9;12) in acute lymphocytic leukemia and other hematological malignancies: report from a dic(9;12) study group. |
| Behrendt H, Charrin C, Gibbons B, Harrison CJ, Hawkins JM, Heerema NA, Horschler-Bötel B, Huret JL, Laï JL, Lampert F |
| Leukemia : official journal of the Leukemia Society of America, Leukemia Research Fund, U.K. 1995 ; 9 (1) : 102-106. |
| PMID 7845002 |
| Lack of association between abnormalities of the chromosome 9 short arm and either lymphomatous features or T cell phenotype in childhood acute lymphocytic leukemia. |
| Carroll AJ, Castleberry RP, Crist WM |
| Blood. 1987 ; 69 (3) : 735-738. |
| PMID 3493041 |
| Lymphoblastic leukemia with lymphomatous features associated with abnormalities of the short arm of chromosome 9. |
| Chilcote RR, Brown E, Rowley JD |
| The New England journal of medicine. 1985 ; 313 (5) : 286-291. |
| PMID 3925340 |
| Review of alterations of the cyclin-dependent kinase inhibitor INK4 family genes p15, p16, p18 and p19 in human leukemia-lymphoma cells. |
| Drexler HG |
| Leukemia : official journal of the Leukemia Society of America, Leukemia Research Fund, U.K. 1998 ; 12 (6) : 845-859. |
| PMID 9639410 |
| Association of chromosome arm 9p abnormalities with adverse risk in childhood acute lymphoblastic leukemia: A report from the Children's Cancer Group. |
| Heerema NA, Sather HN, Sensel MG, Liu-Mares W, Lange BJ, Bostrom BC, Nachman JB, Steinherz PG, Hutchinson R, Gaynon PS, Arthur DC, Uckun FM |
| Blood. 1999 ; 94 (5) : 1537-1544. |
| PMID 10477677 |
6. Citation
| This paper should be referenced as such : |
| Heerema, NA |
| 9p rearrangements in ALL |
| Atlas Genet Cytogenet Oncol Haematol. 1999;3(3):153-154. |
| Free journal version : [ pdf ] [ DOI ] |
| On line version : http://atlasgeneticsoncology.usal.es/classic/Anomalies/9prearrALLID1156.html |
7. External links
| REVIEW articles | automatic search in PubMed |
| Last year articles | automatic search in PubMed |
| All articles | automatic search in PubMed |
| © Atlas of Genetics and Cytogenetics in Oncology and Haematology | indexed on : Wed Nov 28 16:16:32 CET 2018 |
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