| Clinics and Pathology |
| Disease | chronic lymphoproliferative disorder (CLD) |
| Phenotype / cell stem origin | disease affecting mature B-cells; immunophenotypically, B-PLL is characterized by reactivity with pan B-cell markers CD19, CD20 and CD24; B-PLL cells are distinct from B-CLL cells in that they express bright surface immunoglobulin, unfrequently express CD5, fail to form rosettes with mouse erythrocytes and react strongly with FMC7; expression of CD22 is often observed whereas CD23 is usually not expressed |
| Epidemiology | rare disease; slight male predominance with median age of 69 years |
| Clinics | patients often present with advanced stage disease. B-PLL is characterized by high white blood cell counts and splenomegaly without adenopathy; |
| Prognosis | evolution: this disease is always progressive; prognosis: poor response to therapy is often observed; median survival is 3 years |
| Cytogenetics |
| Cytogenetics Morphological | Few studies focused on B-PLL; the use of B-cell mitogens might increase the detection rate of cytogenetic changes; the most frequent aberrations involve chromosomes 14, 6 and 1; 14q+ changes are the most commonly observed and are often the consequence of a translocation t(11;14)(q13;q32); structural abnormalities of chromosome 6 are primary or secondary; deletion 6q, as well as translocation t(6;12)(q15;p13) are described; structural aberrations of chromosome 1 involve both p and q arms; trisomy 12 represents a secondary change in this disease; finally, i(17)(q10), as well as telomeric associations have been reported; karyotypic evolution has been documented in some cases and seems to be associated with disease progression. |
| Genes involved and Proteins |
| Note | Little is known about underlying genetic mechanisms in B-PLL. Immunoglobulin gene rearrangements are always observed. BCL-1 gene is involved in some cases bearing t(11;14)(q13;q32), with breakpoints located centrometric to the major translocation cluster. Overall, abnormalities of P53 occur in 75% cases, representing the highest reported frequency in B-cell malignancies. No CDKNL-2 or RB1 gene involvement has been documented so far. C-MYC rearrangement has been described in PLL. |
| To be noted |
| T-cell prolymphocytic leukaemia also exists and account for 1/4 of cases of PLL |
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| Contributor(s) |
| Written | 10-1998 | Lucienne Michaux |
| Department of Hematology and Center for Human Genetics Cliniques Universitaires Saint Luc Avenue Hippocrate 10 1200 Brussels, Belgium |
| Citation |
| This paper should be referenced as such : |
| Michaux L . B-cell prolymphocytic leukemia (B-PLL). Atlas Genet Cytogenet Oncol Haematol. October 1998 . URL : http://AtlasGeneticsOncology.org/Genes/BPLL.html |
| © Atlas of Genetics and Cytogenetics in Oncology and Haematology | indexed on : Wed Sep 24 21:05:27 2008 |
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