|| 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).
|Phenotype / cell stem origin
|| Chronic B-cell lymphoproliferation|
|Epidemiology|| 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.|
|Clinics|| Often a slow evolutive disease.|
|Prognosis|| 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.|
|Cytogenetics Morphological|| The t(14;19)(q32.3;q13.2) is reciprocal and results in 14q+ and a 19q- derivative chromosomes.|
|Cytogenetics Molecular|| FISH is useful for identifying variant translocations.|
|Additional anomalies|| 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.|
|Variants|| 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 t(14;19)(q32;q13)-positive small B-cell leukaemia: a clinicopathologic and cytogenetic study of seven cases.|
| Huh YO, Abruzzo LV, Rassidakis GZ, Parry-Jones N, Schlette E, Brito-Bapabulle V, Matutes E, Wotherspoon A, Keating MJ, Medeiros LJ, Catovsky D.|
| Br J Haematol. 2007 Jan;136(2):220-8.|
| A comprehensive genetic and histopathologic analysis identifies two subgroups of B-cell malignancies carrying a t(14;19)(q32;q13) or variant BCL3-translocation.|
| Mart'n-Subero JI, Ibbotson R, Klapper W, Michaux L, Callet-Bauchu E, Berger F, Calasanz MJ, De Wolf-Peeters C, Dyer MJ, Felman P, Gardiner A, Gascoyne RD, Gesk S, Harder L, Horsman DE, Kneba M, Kuppers R, Majid A, Parry-Jones N, Ritgen M, Salido M, Sole F, Thiel G, Wacker HH, Oscier D, Wlodarska I, Siebert R.|
| Leukemia. 2007 Jul;21(7):1532-44.|
| t(14;19)/BCL3 rearrangements in lymphoproliferative disorders: a review of 23 cases.|
| Michaux L, Dierlamm J, Wlodarska I, Bours V, Van den Berghe H, Hagemeijer A.|
| Cancer genetics and cytogenetics. 1997 ; 94 (1) : 36-43.|
| BCL3 rearrangement and t(14;19)(q32;q13) in lymphoproliferative disorders.|
| Michaux L, Mecucci C, Stul M, Wlodarska I, Hernandez JM, Meeus P, Michaux JL, Scheiff JM, Noel H, Louwagie A, Criel A, Boogaerts M, Van Orshoven A, Cassiman JJ, Van Den Berghe H.|
| Genes, chromosomes & cancer. 1996 ; 15 (1) : 38-47.|
| Splenic small B-cell lymphoma with IGH/BCL3 translocation.|
| Soma LA, Gollin SM, Remstein ED, Ketterling RP, Flynn HC, Rajasenan KK, Swerdlow SH.|
| Hum Pathol. 2006 Feb;37(2):218-30. (Review)|