B-cell non Hodgkin lymphoma of the low to intermediate grade

annual incidence 5/10⁶; median age: 65 yrs

advanced disease

median survival: 3 to 4 yrs

chronic lymphoproliferative disorder affecting mature B-cells

rare disease; median age 70 yrs

patients often present with advanced stage disease

median survival: 3 yrs

proliferation involving plasma cells

rare disorder

median survival is less than a yr

chronic B-cell lymphoproliferation

rare disorder; median age: 70 yrs

relatively benign clinical course

80% 5-yr survival

chronic lymphocytic leukaemia

chronic B-cell lymphoproliferation

annual incidence 30/10⁶; median age: 60-80 yrs

often a slow evolutive disease

highly variable according to the staging: from staging A: survival not reduced compared to age matched population, to staging C: median survival of 2 yrs

malignant plasma cell proliferation (terminally differenciated B-cell)

annual incidence: 30/10⁶; median age: 60 yrs

median survival: 3 yrs.

t(11;14) has earlier been thought to be the hallmark of the mantle cell lymphoma; actually, the frequency of t(11;14) is: 50-70% in mantle cell lymphoma, 10-20% in B-prolymphocytic leukaemia, in plasma cell leukaemia, and in splenic lymphoma with villous lymphocytes, and 2-5% in chronic lymphocytic leukaemia, and in multiple myeloma.

in particular interphase cytogenetics, are relevant in these diseases with an usually low mitotic index

sole anomaly in only 10% of cases; part of a complex karyotype in 2/3 of cases; numerous recurrent anomalies found conjointly (which is the primary?), particularly: +3, +7, del(9p), +18, + mar, found in about 10% of cases each; other: del(1p), del(6q), del(7q), -8, +12, del(13q), del(17p)

three way complex t(11;14; Var) exist and showed that the crucial event lies on der(14)

5 BCL1 translocated on chromosome 14 near JH (junctions genes of IgH) and C in 3; the breakpoint in BCL1 is in MTC (major translocation cluster), centromeric to the gene (in 5), in 80% of cases, or dispersed in mTC1, 2, or 3 in 5of the gene or in the 3 untranslated region of exon 5.no fusion protein, but promoter exchange; the immunoglobulin gene enhancer stimulates the expression of BCL1

overexpression of BCL1 accelerates passage through the G1 phase.

IGH/CCND1 IGH (14q32.33) CCND1 (11q13.3) M ins(14;11)(q32;q13q13) t(11;14)(q13;q32) t(11;19;14)(q13;q13;q32) t(14;16)(q32;q23) t(14;20)(q32;q12) t(1;11;14)(q32;q13;q32) t(3;14;11)(q21;q32;q13) t(4;14)(p16;q32) t(6;14)(p21;q32)

t(11;14) is mainly found in mantle cell lymphoma, but also in B-prolymphocytic leukaemia, in plasma cell leukaemia, in splenic lymphoma with villous lymphocytes, in chronic lymphocytic leukaemia, and in multiple myeloma, herein briefly described; all these diseases involve a B-lineage lymphocyte.