del(13q) in chronic lymphoproliferative diseases

1999-11-01   Antonio Cuneo 

1.Hematology Section, Dept. Of Biomedical Sciences, University of Ferrara, 44100 Ferrara Italy

Clinics and Pathology

Disease

atypical CLL, including the CLL/PL (prolymphocytic leukemia) or CLL mixed-cell-type variant by FAB criteria

Phenotype stem cell origin

virgin CD5+ recirculating B-cell

Epidemiology

del(13q) is found in approximately 10-15% of all CLLs

Clinics

the clinical course may be more aggressive than in typical CLL, depending on stage at presentation and % of prolymphocytes

Disease

splenic lymphoma with villous lymphcytes

Phenotype stem cell origin

chronic proliferation originating from the marginal zone B-lymphocytes

Epidemiology

the disorder appears to be relatively rare, but it is probably underdiagnosed

Clinics

the clinical course is indolent

Disease

leukemic mantle cell lymphoma

Note

the majority of mantle cell lymphomas show peripheral blood (PB) involvement at diagnosis or at disease evolution; there is a disease variant presenting as a de novo leukemic condition, presenting heterogeneous cytological features with PB and BM lymphocytosis, without adenopathy, with or withour splenomegaly; some of these cases may fulfill the FAB criteria for the diagnosis of atypical CLL; because these cases usually carry the t(11;14)(q13;q32) and a mantle-cell phenotype, they have also been referred to as "mantle cell leukemia": it is reasonable to assume that the transformation of a mantle cell may give rise to a spectrum of diseases ranging from the classical lymphomatous form of MCL to an overt leukemic condition, as is the case with small lymphocytic lymphoma and chronic lymphocytic leukemia

Phenotype stem cell origin

proliferation of cells of follicle mantle lineage (CD5/CD19/CD22 positive, CD23 negative, bright sIg expression)

Bibliography

Pubmed IDLast YearTitleAuthors
93694291997Chromosome aberrations in atypical chronic lymphocytic leukemia: a cytogenetic and interphase cytogenetic study.Bigoni R et al
10406898199913q14 deletion in non-Hodgkin's lymphoma: correlation with clinicopathologic features.Cuneo A et al
95634921998Molecular cytogenetic analysis in splenic lymphoma with villous lymphocytes: frequent allelic imbalance of the RB1 gene but not the D13S25 locus on chromosome 13q14.García-Marco JA et al
104829841999Cyclin D1 overexpression allows identification of an aggressive subset of leukemic lymphoproliferative disorder.Levy V et al
86394561996Mantle cell leukaemia?Neilson JR et al
95836871998Expressed sequences as candidates for a novel tumor suppressor gene at band 13q14 in B-cell chronic lymphocytic leukemia and mantle cell lymphoma.Stilgenbauer S et al

Summary

Note

a spectrum of B-cell chronic lymphoproliferative disorders (CLD) may carry a chromosome 13q deletion; among these, three forms other than chronic lymphocytic leukemia (CLL)were identified by the FAB group which may frequently carry a 13q- chromosome: atypical CLL, splenic lymphoma with villous lymphocytes, corresponding to splenic marginal zone B-cell lymphoma, and mantle cell lymphoma (MCL) in leukemic phase
Atlas Image
del(13q)  Top row: Partial karyotypes with various 13q deletions u2013 Courtesy Adriana Zamecnikova. Middle row: clones dJ1154H7 (top) and dJ1013C9 (bottom) for 13q14 deletions, in normal cells - Courtesy Mariano Rocchi. Bottom row: Hybridization with the LSI 13q14 (red signal) and the subtelomeric probe (green signal) on normal metaphase (A) and showing the presence of 1 green/2 red signal (13q34 deletion) (B) and the two green/one red signal (interstitial deletion involving the 13q14 region) (C). Hybridization with LSI D13S319/LSI 13q34/ CEP 12 multi-color probe showing deletion involving 13q34 (missing 1 blue signal) (D) u2013 Courtesy Adriana Zamecnikova.

Citation

Antonio Cuneo

del(13q) in chronic lymphoproliferative diseases

Atlas Genet Cytogenet Oncol Haematol. 1999-11-01

Online version: http://atlasgeneticsoncology.org/haematological/2065/del(13q)-in-chronic-lymphoproliferative-diseases

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