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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

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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 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


Molecular cytogenetic abnormalities in multiple myeloma and plasma cell leukemia measured using comparative genomic hybridization.
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PMID 9115968
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PMID 8182933
The clinical significance of cytogenetic studies in 100 patients with multiple myeloma, plasma cell leukemia, or amyloidosis.
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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.
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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.
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PMID 7537117
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Cancer genetics and cytogenetics. 1995 ; 83 (1) : 25-27.
PMID 7656199
Correlations between karyotype and cytologic findings in multiple myeloma.
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PMID 8759879


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 :

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


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