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B-cell prolymphocytic leukemia (B-PLL) (published in 1998)

Written1998-10Lucienne Michaux
Department of Hematology, Center for Human Genetics Cliniques Universitaires Saint Luc Avenue Hippocrate 10 1200 Brussels, Belgium

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ICD-Morpho 9833/3 B-cell prolymphocytic leukaemia
Atlas_Id 1860

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;
bone marrow infiltration pattern is either diffuse or mixed;
blood data: elevated white blood cell counts with prolymphocytes representing more than 55% of the circulating lymphoid cells;
anemia and thrombocytopenia may be observed

Prognosis evolution: this disease is always progressive; prognosis: poor response to therapy is often observed; median survival is 3 years


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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This paper should be referenced as such :
Michaux, L
B-cell prolymphocytic leukemia (B-PLL)
Atlas Genet Cytogenet Oncol Haematol. 1999;3(1):24-25.
Free journal version : [ pdf ]   [ DOI ]
On line version :

External links

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