title: B-cell prolymphocytic leukemia: a specific subgroup of mantle cell lymphoma.
abstract: B-cell prolymphocytic leukemia (B-PLL) is a rare mature B-cell malignancy that may be hard to distinguish from mantle cell lymphoma (MCL) and chronic lymphocytic leukemia (CLL). B-PLL cases with a t(11;14) were redefined as MCL in the World Health Organization 2008 classification. We evaluated 13 B-PLL patients [7 being t(11;14)-positive (B-PLL+) and 6 negative (B-PLL-)] and compared them with MCL and CLL patients. EuroFlow-based immunophenotyping showed significant overlap between B-PLL+ and B-PLL-, as well as between B-PLL and MCL, whereas CLL clustered separately. Immunogenotyping showed specific IGHV gene usage partly resembling MCL. Gene expression profiling showed no separation between B-PLL+ and B-PLL- but identified 3 subgroups. One B-PLL subgroup clustered close to CLL and another subgroup clustered with leukemic MCL; both were associated with prolonged survival. A third subgroup clustered close to nodal MCL and was associated with short survival. Gene expression profiles of both B-PLL+ and B-PLL- showed best resemblance with normal immunoglobulin M-only B-cells. Our data confirm that B-PLL+ is highly comparable to MCL, indicate that B-PLL- also may be considered as a specific subgroup of MCL, and suggest that B-PLL is part of a spectrum, ranging from CLL-like B-PLL, to leukemic MCL-like B-PLL, to nodal MCL-like B-PLL.
authors.affiliation: Department of Immunology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands;
authors.forename: Vincent H J
authors.initials: VH
authors.lastname: van der Velden
authors.affiliation: Department of Immunology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands;
authors.forename: Patricia G
authors.initials: PG
authors.lastname: Hoogeveen
authors.affiliation: Delft Bioinformatics Laboratory, Faculty of Electrical Engineering, Mathematics and Computer Science, Delft University of Technology, Delft, The Netherlands;
authors.forename: Dick
authors.initials: D
authors.lastname: de Ridder
authors.affiliation: Department of Immunology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands;
authors.forename: Magdalena
authors.initials: M
authors.lastname: Schindler-van der Struijk
authors.affiliation: Department of Immunology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands;
authors.forename: Menno C
authors.initials: MC
authors.lastname: van Zelm
authors.affiliation: Department of Hematology, Erasmus University Medical Center Rotterdam, Rotterdam, The Netherlands;
authors.forename: Mathijs
authors.initials: M
authors.lastname: Sanders
authors.affiliation: Second Department of Medicine, University Hospital of Schleswig Holstein, Campus Kiel, Kiel, Germany;
authors.forename: Dennis
authors.initials: D
authors.lastname: Karsch
authors.affiliation: Department of Clinical Genetics and.
authors.forename: H Berna
authors.initials: HB
authors.lastname: Beverloo
authors.affiliation: Department of Pathology, Erasmus University Medical Center Rotterdam, Rotterdam, The Netherlands; and.
authors.forename: King
authors.initials: K
authors.lastname: Lam
authors.affiliation: Cancer Research Center (Instituto de Biología Molecular y Celular del Cancer-Consejo Superior de Investigaciones Científicas), Department of Medicine and Cytometry Service (Nucleus), University of Salamanca, and Institute of Biomedical Research of Salamanca, Salamanca, Spain.
authors.forename: Alberto
authors.initials: A
authors.lastname: Orfao
authors.affiliation: Department of Hematology, Erasmus University Medical Center Rotterdam, Rotterdam, The Netherlands;
authors.forename: Pieternella J
authors.initials: PJ
authors.lastname: Lugtenburg
authors.affiliation: Second Department of Medicine, University Hospital of Schleswig Holstein, Campus Kiel, Kiel, Germany;
authors.forename: Sebastian
authors.initials: S
authors.lastname: Böttcher
authors.affiliation: Department of Immunology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands;
authors.forename: Jacques J M
authors.initials: JJ
authors.lastname: van Dongen
authors.affiliation: Department of Immunology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands;
authors.forename: Anton W
authors.initials: AW
authors.lastname: Langerak
authors.affiliation: Department of Hematology, Erasmus University Medical Center Rotterdam, Rotterdam, The Netherlands;
authors.forename: Mies
authors.initials: M
authors.lastname: Kappers-Klunne
authors.affiliation: Department of Hematology, Erasmus University Medical Center Rotterdam, Rotterdam, The Netherlands;
authors.forename: Kirsten
authors.initials: K
authors.lastname: van Lom
mesh_terms: D000328:Adult; D000368:Aged; D016175:B-Lymphocyte Subsets / Q000276:immunology; D005260:Female; D015326:Gene Rearrangement, B-Lymphocyte, Heavy Chain; D050438:Genes, Immunoglobulin Heavy Chain; D006801:Humans; D016130:Immunophenotyping; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000145:classification / Q000235:genetics / Q000276:immunology; D054403:Leukemia, Prolymphocytic, B-Cell / Q000145:classification* / Q000235:genetics / Q000276:immunology; D020522:Lymphoma, Mantle-Cell / Q000145:classification* / Q000235:genetics / Q000276:immunology; D008297:Male; D008875:Middle Aged; D059467:Transcriptome