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. |
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 |
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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 |
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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 |
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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 |
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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 |
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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 |
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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 |
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