Chronic lymphocytic leukemia/small lymphocytic lymphoma (B-CLL/SLL); most are the atypical form (at times reported as variant CLL, or transformed CLL), because of an atypical morphology and phenotype (Michaux et al., 1996; Michaux et al., 1997), but also for the presence of lymphocytosis (Soma et al., 2006), the frequency of lymphadenopathy (Huh et al., 2007), young age (median 50-60 years), male predominence (about 2M/1F), and an aggressive course of the disease (Michaux et al., 1997).
Other diseases: marginal zone lymphoma (MZL), splenic marginal zone lymphoma (SMZL), diffuse large B-cell lymphoma (DLBCL), mantle cell lymphoma, low grade B-cell non Hodgkin lymphoma (NHL) not otherwise specified, aggressive B-cell NHL, and even one case of biphenotypic (B/M) acute leukemia.
A recent study reascertained and split the entity into the following subgroups herein below described; the accuracy of this proposal remains to be confirmed by further studies, inasmuch as there is a real problem of classification of t(14;19)(q32;q13) in chronic B-cell lymphoproliferative disorders, as has been pointed out (Soma et al., 2006; Huh et al., 2007):
"7q rearrangements cluster": 15% of cases: diagnosis of MZL/SMZL or aggressive B-cell NHL mostly. Medium complexity of the karyotype. IgVH mutated. Aggressive diseases. Median age 65 (49-85).
"Deletion 17p cluster": 10% of cases: diagnosis of CLL frequent. Complex karyotypes. IgVH mutated. Median age 54.
"1q rearrangements, deletions 6q and 13q cluster": 30% of cases: diagnosis of DLBCL or MZL and MZL in transformation, less often, CLL. Complex karyotypes. IgVH mutated. Aggressive diseases. Median age 65 (27-92).
"Trisomy 12 cluster": 45% of cases: diagnosis of CLL in most cases, low complexity of the karyotype, IgVH mutation rate low. This is the only cluster with an unequal sex ratio: 14M/4F; median age 65 (35-95).

Chronic B-cell lymphoproliferation

103 published cases (40 reviewed in Soma et al., 2006; 7 cases in Huh et al., 2007; and 56 cases in Martin-Subero et al., 2007). Annual incidence 30/10⁶. The t(14;19) occurs in less than 0.2 % of B-cell malignancies.

Often a slow evolutive disease.

Highly variable according to the staging: from staging A: where the survival is not reduced compared to age matched population, to staging C: with a median survival of 2 yrs. t(14;19) is often associated with rapidly progressive disease, and overall prognosis is poor compared to the expected survival in chronic lymphocytic leukemia and low-grade B-cell lymphoma. The prognosis has to be reascertained according to new (or further) sub-classification of the the disease.

The t(14;19)(q32.3;q13.2) is reciprocal and results in 14q+ and a 19q- derivative chromosomes.

FISH is useful for identifying variant translocations.

t(14;19) is rarely the sole cytogenetic aberration. Trisomy 12 is the most frequent associated abnormality, and is observed in 50% of cases. del(6q), del(7q), del(13q), del(17p) and additional 14q32 rearrangements can be found. Other chromosomes involved in structural aberrations are 1q, 3p, 3q, 6p, 7q, 11q, 12p.

t(2;19)(p12;q13) IGK/BCL3 and t(19;22)(q13;q11) BCL3/IGL, variants of the t(14;19)(q32;q13) IGH/BCL3, have been described; they are found in the same proportions as for the variants of the t(8;14)(q24;q32).

The breakpoint is located in the 5 untranslated region of the BCL3 gene. BCL3 is juxtaposed to the immunoglobulin heavy chain gene locus on chromosome 14 (often in the switch alpha region) in a "head-to-head" configuration.

IGK/BCL3 IGK (14q32.33) BCL3 (19q13.32) M t(2;19)(p11;q13)

The t(14;19)(q32.3;q13.3) -as well as the variants t(2;19) and t(19;22)- is a recurrent translocation found in patients with chronic B-cell lymphoproliferative disorders.
This abnormality is cytogenetically identical but molecularly distinct from the t(14;19)(q32;q13) with IGH and CEBPA (or CEBPG) involvements, seen in acute B-cell lymphoblastic leukaemia.