Review on Lymphoplasmacytic lymphoma, with data on clinics, and the genes involved

Castoldi, GL

Cuneo, A

Atlas of Genetics and Cytogenetics in Oncology and Haematology

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Leukaemia Section 
Short Communication 
 
Atlas Genet Cytogenet Oncol Haematol. 2000; 4(2)  
 
85 
Atlas of Genetics and Cytogenetics 
in Oncology and Haematology 
OPEN ACCESS JOURNAL AT INIST-CNRS 
Lymphoplasmacytic lymphoma 
Antonio Cuneo, Gian Luigi Castoldi 
Hematology Section, Department of Biomedical Sciences, University of Ferrara, Corso Giovecca 203, 
Ferrara, Italy (AC) 
 
Published in Atlas Database: May 2000 
Online updated version : http://AtlasGeneticsOncology.org/Anomalies/LPLID2074.html 
DOI: 10.4267/2042/37625 
This work is licensed under a Creative Commons Attribution-Noncommercial-No Derivative Works 2.0 France Licence. 
© 2000 Atlas of Genetics and Cytogenetics in Oncology and Haematology 
 
Clinics and pathology 
Disease 
Lymphoplasmacytic lymphoma (LPL) includes most 
cases of Waldenstrom's macroglobulinemia and 
consists of a diffuse proliferation of small lymphocytes, 
plasmacytoid lymphocytes and plasma cells; the typical 
histologic features of small lymphocytic lymphoma, 
mantle cell lymphoma and marginal zone lymphoma 
must be absent. 
Phenotype/cell stem origin 
B-cell antigens are positive; whereas CD5 and, usually, 
CD23 test negative; the cells express surface and 
cytoplasmic IgM and are IgD-. CD10 is negative, 
whereas a proportion of cases have faint CD25 and 
CD11c expression; the postulated normal counterpart is 
a B-lymphocyte differentiating into an IgM-secreting 
plasma cell. 
Cytogenetics 
Note 
The number of cases studied is small and the 
interpretation of data must take into account possible 
variability of histologic classification. 
Cytogenetics morphological 
The t(9;14)(p13;q32), fusing the IgH gene with the 
BSAP (B-cell specific activator protein), also called 
PAX5, was found in 50% of the cases in a study; this
balanced translocation cannot be viewed as specific for 
this lymphoma, having been reported in rare marginal 
zone lymphomas and large cell lymphomas. 
Other recurrent anomalies include 14q32 translocatins 
with 8q24 (t(8;14)(q24;q32)), 18q21 
(t(14;18)(q32;q21)), trisomies of chromosomes 3 (+3) 
and 12 (+12) and an isochromosome 6p (i(6p)) 
References 
Offit K, Parsa NZ, Filippa D, Jhanwar SC, Chaganti RS. 
t(9;14)(p13;q32) denotes a subset of low-grade non-Hodgkin's 
lymphoma with plasmacytoid differentiation. Blood. 1992 Nov 
15;80(10):2594-9 
Harris NL, Jaffe ES, Stein H, Banks PM, Chan JK, Cleary ML, 
Delsol G, De Wolf-Peeters C, Falini B, Gatter KC. A revised 
European-American classification of lymphoid neoplasms: a 
proposal from the International Lymphoma Study Group. 
Blood. 1994 Sep 1;84(5):1361-92 
Panayiotidis P, Kotsi P. Genetics of small lymphocyte 
disorders. Semin Hematol. 1999 Apr;36(2):171-7 
This article should be referenced as such: 
Cuneo A, Castoldi GL. Lymphoplasmacytic lymphoma. Atlas 
Genet Cytogenet Oncol Haematol. 2000; 4(2):85. 


Lymphoplasmacytic lymphoma

http://documents.irevues.inist.fr/bitstream/2042/37625/3/05-2000-LPLID2074.pdf

Lymphoplasmacytic lymphoma

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