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B-cell prolymphocytic leukemia (B-PLL)

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

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Identity

ICD-Topo C420,C421,C424 BLOOD, BONE MARROW, & HEMATOPOIETIC SYS
ICD-Morpho 9833/3 B-cell prolymphocytic leukaemia
Atlas_Id 2033

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

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

Bibliography

Proposals for the classification of chronic (mature) B and T lymphoid leukaemias. French-American-British (FAB) Cooperative Group.
Bennett JM, Catovsky D, Daniel MT, Flandrin G, Galton DA, Gralnick HR, Sultan C
Journal of clinical pathology. 1989 ; 42 (6) : 567-584.
PMID 2738163
 
Burkitt-like mutations in the c-myc gene locus in prolymphocytic leukemia.
Brennscheidt U, Eick D, Kunzmann R, Martens U, Kiehntopf M, Mertelsmann R, Herrmann F
Leukemia : official journal of the Leukemia Society of America, Leukemia Research Fund, U.K. 1994 ; 8 (5) : 897-902.
PMID 8182948
 
Translocation t(11;14)(q13;q32) in chronic lymphoid disorders.
Brito-Babapulle V, Ellis J, Matutes E, Oscier D, Khokhar T, MacLennan K, Catovsky D
Genes, chromosomes & cancer. 1992 ; 5 (2) : 158-165.
PMID 1381952
 
Molecular cytogenetic analysis of RB-1 deletions in chronic B-cell leukemias.
Döhner H, Pilz T, Fischer K, Cabot G, Diehl D, Fink T, Stilgenbauer S, Bentz M, Lichter P
Leukemia & lymphoma. 1994 ; 16 (1-2) : 97-103.
PMID 7696936
 
Molecular cloning of a t(11;14)(q13;q32) translocation breakpoint centromeric to the BCL1-MTC.
Galiègue-Zouitina S, Collyn-d'Hooghe M, Denis C, Mainardi A, Hildebrand MP, Tilly H, Bastard C, Kerckaert JP
Genes, chromosomes & cancer. 1994 ; 11 (4) : 246-255.
PMID 7533528
 
Telomeric associations in a patient with B-cell prolymphocytic leukaemia.
Howell RT, Kitchen C, Standen GR
Genes, chromosomes & cancer. 1993 ; 7 (2) : 116-118.
PMID 7687453
 
p53 abnormalities in B-cell prolymphocytic leukemia.
Lens D, De Schouwer PJ, Hamoudi RA, Abdul-Rauf M, Farahat N, Matutes E, Crook T, Dyer MJ, Catovsky D
Blood. 1997 ; 89 (6) : 2015-2023.
PMID 9058723
 
Chromosome abnormalities in B-cell prolymphocytic leukemia: a study of nine cases.
Pittman S, Catovsky D
Cancer genetics and cytogenetics. 1983 ; 9 (4) : 355-365.
PMID 6603259
 
Possible specific chromosome change in prolymphocytic leukemia.
Sadamori N, Han T, Minowada J, Bloom ML, Henderson ES, Sandberg AA
Blood. 1983 ; 62 (4) : 729-736.
PMID 6882921
 
CDKN2 gene deletion is not found in chronic lymphoid leukaemias of B- and T-cell origin but is frequent in acute lymphoblastic leukaemia.
Schröder M, Mathieu U, Dreyling MH, Bohlander SK, Hagemeijer A, Beverloo BH, Olopade OI, Stilgenbauer S, Fischer K, Bentz M
British journal of haematology. 1995 ; 91 (4) : 865-870.
PMID 8547131
 

Citation

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 : http://AtlasGeneticsOncology.org/Anomalies/BPLLID2033.html


Other genes implicated (Data extracted from papers in the Atlas) [ 3 ]

Genes CCND1 IGH ABCC1

External links

COSMICHisto = - Site = haematopoietic_and_lymphoid_tissue (COSMIC)
arrayMapTopo ( C42) Morph ( 9833/3) - arrayMap (UZH-SIB Zurich)  [auto + random 100 samples .. if exist ]   [tabulated segments]
 
 
Disease databaseB-cell prolymphocytic leukemia (B-PLL)
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


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