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Plasma cell leukemia (PCL)

Written1997-10Lucienne Michaux
Department of Hematology and Center for Human Genetics Cliniques Universitaires Saint Luc Avenue Hippocrate 10 1200 Brussels, Belgium

(Note : for Links provided by Atlas : click)

Identity

ICD-Topo C420,C421,C424 BLOOD, BONE MARROW, & HEMATOPOIETIC SYS
ICD-Morpho 9733/3
Atlas_Id 2040

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
Phenotype / cell stem origin proliferation involving plasma cell expressing cytoplasmic immunoglobulin, CD38, plasma cell antigene 1; a minority of cells express CD10, HLA-DR, and CD20; the nature of the clonogenic cell in multiple myeloma is unknown; the presence of multiple hematopoietic surface antigenes on malignant plasma cells suggests its origin from a pluripotent stem cell
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.
Clinics patients with primary PCL have a greater incidence of hepatosplenomegaly and lymphadenopathy, and fewer lytic bone lesions; blood data: these data are similar to those of multiple myeloma, except that there are circulating plasma cells: patients with PCL have more than 20% plasma cells in their peripheral blood and an absolute plasma cell count equal or above 2000/mm3; additionnally, patients with primary PCL have higher platelets counts and smaller M components compared to patients with secondary PCL
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; prognosis: the overall survival is short (few months).

Cytogenetics

Cytogenetics Morphological Cytogenetic aberrations are detected more frequently in PCL than in multiple myeloma; the percentage of abnormal cases varies in different series but seems to be more than 50%; the overall pattern of cytogenetic changes is very similar to the pattern observed in multiple myeloma; numerical changes and/or structural aberrations have been described; in large series, hyperdiploidy is observed in 61 to 68% of cases, whereas pseudodiploidy and hypodiploidy occur in 9 to 20 and 10 to 30% of patients, respectively; monosomy 13 and trisomy 9 are the most frequent numerical abnormalities; hypodiploidy is more common in PCL than in myeloma. Apart from chromosome 9, gains also involve chromosomes 3, 5, 7, 11, 15, and 19, whereas losses also involve chromosome X and Y; structural aberrations mainly involve chromosome 14, with 14q+ resulting from translocation t(11;14)(q13;q32) or other changes (e.g. Burkitt's translocations); chromosomes 16 (p or q), 1 (p or q), 19 (p or q), 6q, 17q, 2p and 7q might also be involved.
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.

Genes involved and Proteins

Note Analysis of DNA content of plasma cells demonstrates abnormalities in almost all patients; in addition, rearrangements and amplification of the proto-oncogene C-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

Bibliography

Molecular cytogenetic abnormalities in multiple myeloma and plasma cell leukemia measured using comparative genomic hybridization.
Avet-Loiseau H, Andree-Ashley LE, Moore D 2nd, Mellerin MP, Feusner J, Bataille R, Pallavicini MG
Genes, chromosomes & cancer. 1997 ; 19 (2) : 124-133.
PMID 9172003
 
Cytogenetic analysis of 280 patients with multiple myeloma and related disorders: primary breakpoints and clinical correlations.
Calasanz MJ, Cigudosa JC, Odero MD, Ferreira C, Ardanaz MT, Fraile A, Carrasco JL, Solé F, Cuesta B, Gullón A
Genes, chromosomes & cancer. 1997 ; 18 (2) : 84-93.
PMID 9115968
 
Inactivation of tumor suppressor genes, p53 and Rb1, in plasma cell dyscrasias.
Corradini P, Inghirami G, Astolfi M, Ladetto M, Voena C, Ballerini P, Gu W, Nilsson K, Knowles DM, Boccadoro M
Leukemia : official journal of the Leukemia Society of America, Leukemia Research Fund, U.K. 1994 ; 8 (5) : 758-767.
PMID 8182933
 
The clinical significance of cytogenetic studies in 100 patients with multiple myeloma, plasma cell leukemia, or amyloidosis.
Dewald GW, Kyle RA, Hicks GA, Greipp PR
Blood. 1985 ; 66 (2) : 380-390.
PMID 3926026
 
Cellular and molecular genetic features of myeloma and related disorders.
Durie BG
Hematology/oncology clinics of North America. 1992 ; 6 (2) : 463-477.
PMID 1582985
 
Chromosome studies in plasma cell leukemia and multiple myeloma in transformation.
Jonveaux P, Berger R
Genes, chromosomes & cancer. 1992 ; 4 (4) : 321-325.
PMID 1377939
 
Improved cytogenetics in multiple myeloma: a study of 151 patients including 117 patients at diagnosis.
Laï JL, Zandecki M, Mary JY, Bernardi F, Izydorczyk V, Flactif M, Morel P, Jouet JP, Bauters F, Facon T
Blood. 1995 ; 85 (9) : 2490-2497.
PMID 7537117
 
Molecular breakpoints of t(11;14)(q13;q32) in multiple myeloma.
Meeus P, Stul MS, Mecucci C, Cassiman JJ, Van den Berghe H
Cancer genetics and cytogenetics. 1995 ; 83 (1) : 25-27.
PMID 7656199
 
Correlations between karyotype and cytologic findings in multiple myeloma.
Weh HJ, Bartl R, Seeger D, Selbach J, Kuse R, Hossfeld DK
Leukemia : official journal of the Leukemia Society of America, Leukemia Research Fund, U.K. 1995 ; 9 (12) : 2119-2122.
PMID 8609726
 
Multiple myeloma: almost all patients are cytogenetically abnormal.
Zandecki M, Laï JL, Facon T
British journal of haematology. 1996 ; 94 (2) : 217-227.
PMID 8759879
 

Citation

This paper should be referenced as such :
Michaux, L
Plasma cell leukemia (PCL)
Atlas Genet Cytogenet Oncol Haematol. 1997;1(2):74-75.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/PlasmCelID2040.html


Other genes implicated (Data extracted from papers in the Atlas) [ 2 ]

Genes CCND1 IGH

External links

COSMICHisto = - Site = haematopoietic_and_lymphoid_tissue (COSMIC)
arrayMapTopo ( C42) Morph ( 9733/3) - arrayMap (UZH-SIB Zurich)  [auto + random 100 samples .. if exist ]   [tabulated segments]
 
 
Disease databasePlasma cell leukemia (PCL)
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
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