Antonio Cuneo
Dipartimento di Scienze Biomediche - Sezione di Ematologia Università di Ferrara - Via Savonarola, 9 - 44100 FERRARA - ITALY tel. int+39.0532.236978; fax: int+39.0532.212142; e-mail sse@dns.unife.it
February 2000
B-cell NHL include a number of clinicopathologic subsets of lymphoid neoplasms having heterogeneous features. This situation is reflected by variations in the classification systems that were proposed over the last decade. Cytogenetic findings were recognized to help defining a rationale biologic ground for the nosologic classification of lymphomas.
In this table an outlook of the salient cytogenetic entities in this spectrum of disorders is presented; a complete illustration of the cytogenetic profile of each disease is provided in specific cards. Unless otherwise specified the WHO classification system will be used.
Histologic subset and Immunophenotype
Putative cell of origin
Cytogenetic entitiy and corresponding clinical features
Small lymphocytic lymphoma (SLL)
Pan-B+; CD5+; CD23+; CD10-; sIgM+ faint
CD5+ virgin B-cell with germline IgV genes1
del(6)(q21-23) (20-30% of the cases)
Lymphoplasmacytic lymphoma
Pan-B+; CD5-; CD10-; cyIgM+
Peripheral B-lymphocyte transforming into plasma cell with mutated IgV genes and ongoing mutations
t(9;14)(p13;q32) PAX5/IgH (50% of cases)
Follicle centre cell lymphoma
Pan-B+; CD10+/-; CD5-; sIg+
Centrocytes / centroblasts of germinal centre origin with somatic hypermutation of the IgV genes and ongoing mutations (antigen driven stimulation)
t(14;18)(q32;q21) / BCL2 Rearr (70-80% of cases)
Diffuse large cell lymphoma
CD19+; CD22+;
CD10-/+; SIg+
Large transformed B-cells harbouring somatic hypermutation of the Ig genes
(ongoing mutations in some cases)
Burkitt's lymphoma
Pan-B+; TdT-; CD10+; CD5-; sIgM+
Peripheral B-cells that have encountered the antigen and harbours somatic hypermutation of the Ig genes
t(8;14)(q24;q32) or variants /
Burkitt-like lymphoma Pan-B+; TdT-; CD10-/+ CD5-; sIg+
Peripheral B-cells that have encountered the antigen
Mantle cell lymphoma
Pan-B +; CD5+;
CD23-; CD10-/+; sIgM+ bright
CD5+ B-cells of the follicle mantle having germline IgV gene sequences
t(11;14)(q13;q32) / BCL1 Rearr (50-90%4)
Marginal zone B-cell lymphoma (MZBCL)
pan-B+; CD5-/+; CD10-; CD23-; CD11c+/-; cyIg + (40% of the cells), sIgM+ bright; sIgD-)
t(11;18)(q21;q21) / PI2 / MLT fusion (30-50% of the low-grade MALT)
Extra-nodal low-grade MALT lymphoma; indolent disease
t(1;14)(p21;q32)
Extra-nodal MALT lymphoma
del(7)(q22-31) (40% of the cases)
Splenic MZBCL
+3/+3q
(30-70% of the cases)
Nodal, extra-nodal and splenic MZBCL
Legend : +: positive in >90% of the cases; +/-: positive in more than 50% of the cases; -/+: positive in less than 50% of cases; -: positive in <10% of the cases; pan-B markers include CD19; CD20; CD79a; R = rearranged; sIg: surface immunoglobulins; cyIg: cytoplasmic Ig; IgV genes: genes encoding for the variable portion of the Ig.
Comments
Atlas of Genetics and Cytogenetics in Oncology and Haematology
Classification of B-cell non-Hodgkin's lymphomas (NHL) : cytogenetic entities, immunopheneotype and clinical features
Online version: http://atlasgeneticsoncology.org/deep-insight/20012/haematological/2006/css/haematological-explorer/