Splenic lymphoma with villous lymphocytes (SLVL) in 2005

2005-02-01   Xavier Troussard  , Xavier Troussard  

1.Laboratoire dHématologie, CHU de Caen, 14 000 Caen, France
2.Laboratoire Pasteur-Cerba, 95066, Cergy-Pontoise, France (HM)

Clinics and Pathology

Phenotype stem cell origin

Light chain restriction surface immunoglobulin. Most cases express IgM and IgD. B-cells express CD19+, CD20+, CD22+, CD24+, CD79b+, FMC7+ and DBA44+. Lack expression of CD5 (85%), CD10, CD23, CD103 and CD123.

Epidemiology

In 1987, the term SLVL was introduced; 1-2% of non-Hodgkin lymphomas ; occurs in the elderly (med 70 yrs) ; sex ratio 2M/1F.

Clinics

splenomegaly without hepatomegaly nor enlarged lymph nodes; monoclonal Ig in a third of cases, autoimmune phenomena in 10% of patients, transformation to high grade lymphoma in 10% of cases.
Atlas Image
Peripheral blood lymphocytosis in 75% of patients and villous lymphocytes on peripheral blood smears (Fig 1).

Pathology

Spleen. Nodular replacement of the white pulp with a central core of small lymphocytes and larger cells in the peripheral marginal zone. Invasion of the splenic red pulp is inconstant. Bone marrow morphology showing intrasinusoidal lymphoma cells.

Treatment

Only in symptomatic patients : splenectomy or chemotherapy with purine analogues. Antiviral therapy (IFN) in patients with SLVL and hepatitis C.

Prognosis

Indolent B-cell malignancy with 5-yr survival : 80%; no consensus on adverse prognostic factors: WBC > 30 x 109/l, low lymphocyte count; cases treated with chemotherapy have shorter survival.

Cytogenetics

Atlas Image
t(11;14)(q13;q32) R-Banding (top left); del(7q) (top right); 13q14 allelic loss at the RB1 locus deletion detected by interphase FISH (bottom)

Genes Involved and Proteins

Note
  • del(7q): gene unknown
  • t(11;14)(q13;q32)BCL1 in 11q13 and IgH in 14q32 are involved in 20% of cases, with or without visible (11;14); BCL1 encodes the cyclin D1; role in the cell cycle control (G1 progression and G1/S transition); 5 BCL1 translocated on chromosome 14 near JH, resulting in promoter exchange; the immunoglobulin gene enhancer stimulates the expression of BCL1, and overexpression of BCL1 which accelerates passage through the G1 phase.
  • trisomy 3: gene unknown but region 3q13.q32-q29 over-represented.
  • t(6;14)(p21;q32) cyclin D3 is located on 6p21 and, as CDK6, is implicated in the progression through the G1 phase of the cell cycle.
  • t(2;7)(p12;q21). CDK6 is located on 7q21 and dysregulation of CDK6 gene expression could be contribute to the pathogenesis of SLVL and SMZL.

    • Article Bibliography

    Pubmed IDLast YearTitleAuthors
    118304792002Analysis of the IgV(H) somatic mutations in splenic marginal zone lymphoma defines a group of unmutated cases with frequent 7q deletion and adverse clinical course.Algara P 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
    121107362002Regression of splenic lymphoma with villous lymphocytes after treatment of hepatitis C virus infection.Hermine O et al
    155722162005Splenic marginal zone lymphoma.Oscier D et al
    81362701993Cytogenetic studies in splenic lymphoma with villous lymphocytes.Oscier DG et al
    86524031996Splenic lymphoma with villous lymphocytes: clinical presentation, biology and prognostic factors in a series of 100 patients. Groupe Francais d'Hématologie Cellulaire (GFHC).Troussard X et al

    Citation

    Xavier Troussard ; Xavier Troussard

    Splenic lymphoma with villous lymphocytes (SLVL) in 2005

    Atlas Genet Cytogenet Oncol Haematol. 2005-02-01

    Online version: http://atlasgeneticsoncology.org/haematological/2063/haematological-explorer/gene-fusions-explorer/deep-insight-explorer/

    Historical Card

    1998-10-01 Splenic lymphoma with villous lymphocytes (SLVL) in 2005 by  Jean-Loup Huret,Hossain Mossafa 

    Laboratoire Pasteur-Cerba, 95066, Cergy-Pontoise, France (HM)