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

1998-10-01   Lucienne Michaux  , Lucienne Michaux  

1.Department of Hematology, Center for Human Genetics Cliniques Universitaires Saint Luc Avenue Hippocrate 10 1200 Brussels, Belgium

Clinics and Pathology

Disease

chronic lymphoproliferative disorder (CLD)

Phenotype stem cell 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

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.

Bibliography

No bibliography items were found for this article.

Citation

Lucienne Michaux ; Lucienne Michaux

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

Atlas Genet Cytogenet Oncol Haematol. 1998-10-01

Online version: http://atlasgeneticsoncology.org/haematological/1860/b-cell-prolymphocytic-leukemia-(b-pll)-(published-in-1998)