Cancer Report

Cancer : Plasma cell leukemia (PCL)
Summary
Cancer Name Plasma cell leukemia (PCL)
 
Clinics and Pathology
Disease plasma cell dyscrasia; called primary PCL when it is diagnosed in the leukemic phase, and secondary PCL when there is leukemic transformation of a previously recognized multiple myeloma
Epidemiology rare disorder; approximately 60% of patients have the primary form; affects patients of more than 40 years of age; patients with primary PCL are younger than patients with the secondary PCL; slightly more frequent in men than in women.
Prognosis evolution: this disease is usually progressive; secondary PCL rarely responds to chemotherapy because patients already received alkylating agents and became resistant to them; in the primary form, responses have been observed with melphalan and prednisone; the response rate seems to be higher with combination therapy than with single alkylating agents; the overall survival is short (few months).
 
Related Genes
Gene Symbol RB1
Description From COSMIC: " Analysis of DNA content of plasma cells demonstrates abnormalities in almost all patients; in addition, rearrangements and amplification of the proto-oncogene MYC have been reported, as well as point mutations of NRAS and KRAS genes; molecular rearrangements or point mutations of the tumour suppressor genes RB1 and P53 has been reported; the molecular breakpoint of the translocation t(11;14)(q13;q32) involved the PRAD1 gene in 2 cases "
 
Gene Symbol HRAS
Description From COSMIC:
 
Gene Symbol PTENP1
Description From COSMIC:
 
Gene Symbol BCL1
Description " Analysis of DNA content of plasma cells demonstrates abnormalities in almost all patients; in addition, rearrangements and amplification of the proto-oncogene MYC have been reported, as well as point mutations of NRAS and KRAS genes; molecular rearrangements or point mutations of the tumour suppressor genes RB1 and P53 has been reported; the molecular breakpoint of the translocation t(11;14)(q13;q32) involved the PRAD1 gene in 2 cases "
 
Gene Symbol EGFR
Description From COSMIC:
 
Gene Symbol P53
Description " Analysis of DNA content of plasma cells demonstrates abnormalities in almost all patients; in addition, rearrangements and amplification of the proto-oncogene MYC have been reported, as well as point mutations of NRAS and KRAS genes; molecular rearrangements or point mutations of the tumour suppressor genes RB1 and P53 has been reported; the molecular breakpoint of the translocation t(11;14)(q13;q32) involved the PRAD1 gene in 2 cases "
 
Gene Symbol N-RAS
Description From COSMIC: " Analysis of DNA content of plasma cells demonstrates abnormalities in almost all patients; in addition, rearrangements and amplification of the proto-oncogene MYC have been reported, as well as point mutations of NRAS and KRAS genes; molecular rearrangements or point mutations of the tumour suppressor genes RB1 and P53 has been reported; the molecular breakpoint of the translocation t(11;14)(q13;q32) involved the PRAD1 gene in 2 cases "
 
Gene Symbol SMARCB1
Description From COSMIC:
 
Gene Symbol PIK3CA
Description From COSMIC:
 
Gene Symbol FGFR3
Description From COSMIC:
 
Gene Symbol OSM
Description From Atlas of Genetics and Cytogenetics in Oncology and Haematology:
 
Gene Symbol TOPORS
Description From COSMIC:
 
Gene Symbol BRAF
Description From COSMIC:
 
Gene Symbol K-RAS
Description From COSMIC: " Analysis of DNA content of plasma cells demonstrates abnormalities in almost all patients; in addition, rearrangements and amplification of the proto-oncogene MYC have been reported, as well as point mutations of NRAS and KRAS genes; molecular rearrangements or point mutations of the tumour suppressor genes RB1 and P53 has been reported; the molecular breakpoint of the translocation t(11;14)(q13;q32) involved the PRAD1 gene in 2 case "
 
Gene Symbol CDKN2A
Description From COSMIC:
 
Gene Symbol MYC
Description " Analysis of DNA content of plasma cells demonstrates abnormalities in almost all patients; in addition, rearrangements and amplification of the proto-oncogene MYC have been reported, as well as point mutations of NRAS and KRAS genes; molecular rearrangements or point mutations of the tumour suppressor genes RB1 and P53 has been reported; the molecular breakpoint of the translocation t(11;14)(q13;q32) involved the PRAD1 gene in 2 cases "
 
Cytogenetics
Cytogenetics Molecular Chromosomal changes are detectable by conventional cytogenetic techniques or by FISH; in addition, comparative genomic hybridization showed to be a useful tool in PCL, allowing assessment of regions showing copy number changes.
 
Bibliography
Title Inactivation of tumor suppressor genes, p53 and Rb1, in plasma cell dyscrasias.
Authors Corradini P, Inghirami G, Astolfi M, Ladetto M, Voena C, Ballerini P, Gu W, Nilsson K, Knowles DM, Boccadoro M, et al.
Citations Leukemia. 1994 May;8(5):758-67.
PubMedID 94238897
 
Title Improved cytogenetics in multiple myeloma: a study of 151 patients including 117 patients at diagnosis.
Authors Lai JL, Zandecki M, Mary JY, Bernardi F, Izydorczyk V, Flactif M, Morel P, Jouet JP, Bauters F, Facon T
Citations Blood. 1995 May 1;85(9):2490-7. Review
PubMedID 95244871
 
Title Molecular cytogenetic abnormalities in multiple myeloma and plasma cell leukemia measured using comparative genomic hybridization.
Authors Avet-Loiseau H, Andree-Ashley LE, Moore D 2nd, Mellerin MP, Feusner J, Bataille R, Pallavicini MG
Citations Genes Chromosomes Cancer. 1997 Jun;19(2):124-33
PubMedID 97316016
 
Title Molecular breakpoints of t(11;14)(q13;q32) in multiple myeloma.
Authors Meeus P, Stul MS, Mecucci C, Cassiman JJ, Van den Berghe H
Citations Cancer Genet Cytogenet. 1995 Aug;83(1):25-7.
PubMedID 95384958
 
Title Chromosome studies in plasma cell leukemia and multiple myeloma in transformation.
Authors Jonveaux P, Berger R
Citations Genes Chromosomes Cancer. 1992 Jun;4(4):321-5
PubMedID 92322574
 
Title Cellular and molecular genetic features of myeloma and related disorders.
Authors Durie BG
Citations Hematol Oncol Clin North Am 1992 Apr;6(2):463-77
PubMedID 92259422
 
Title Cytogenetic analysis of 280 patients with multiple myeloma and related disorders:primary breakpoints and clinical correlations.
Authors Calasanz MJ, Cigudosa JC, Odero MD, Ferreira C, Ardanaz MT, Fraile A, Carrasco JL, Sole F, Cuesta B, Gullon A
Citations Genes Chromosomes Cancer. 1997 Feb;18(2):84-93.
PubMedID 97187363
 
Title Correlations between karyotype and cytologic findings in multiple myeloma.
Authors Weh HJ, Bartl R, Seeger D, Selbach J, Kuse R, Hossfeld DK
Citations Leukemia. 1995 Dec;9(12):2119-22.
PubMedID 96112606
 
Title The clinical significance of cytogenetic studies in 100 patients with multiple myeloma, plasma cell leukemia, or amyloidosis.
Authors Dewald GW, Kyle RA, Hicks GA, Greipp PR
Citations Blood. 1985 Aug;66(2):380-90.
PubMedID 85253210
 
Title Multiple myeloma: almost all patients are cytogenetically abnormal.
Authors Zandecki M, Lai JL, Facon T
Citations Br J Haematol. 1996 Aug;94(2):217-27
PubMedID 96326221
 
Source and Citation
Source Atlas of Genetics and Cytogenetics in Oncology and Haematology
Citation Michaux L . Plasma cell leukemia (PCL). Atlas Genet Cytogenet Oncol Haematol. October 1997 .
URL : http://AtlasGeneticsOncology.org/Anomalies/PlasmCel.html
URL http://AtlasGeneticsOncology.org/Anomalies/PlasmCel.html