title: Immunophenotypic and genetic characterization of a CD8 positive mantle cell lymphoma in a patient with concomitant Mycosis fungoides.
abstract: Mantle cell lymphoma (MCL) is immunophenotypically characterized by cell surface co-expression of CD19, CD20, CD5, IgM and FMC7. However, the concomitant presence of other antigens distinctive of a particular leukocyte subset, e.g. T-lymphocytes, is an exceptional finding in MCL. Here, the first case of a blastic MCL in leukaemic phase with aberrant expression of the T-cell associated antigen CD8 occurring in a patient with concomitant Mycosis fungoides is described. Comprehensive immunophenotypic analysis showed that the MCL cells expressed the typical B-lymphocytic markers, were CD5 and CD8 positive, but did not express other T-cell proteins, such as CD2, CD3, CD4, CD7, TCRalphabeta and TCRgammadelta. The MCL cells expressed both CD8alpha and CD8beta chains indicating cell surface presence of CD8alphabeta heterodimers. Intriguingly, expression of the cytotoxic enzymes perforin and granzyme A was detected by RT-PCR. Cytogenetic and molecular genetic analysis of the lymphoma cells confirmed cyclin D1 overexpression secondary to the t(11;14)(q13;32) chromosomal translocation. Furthermore, trisomy 11, trisomy 14 and extra copies of t(11;14) translocated chromosomes were detected in sub clones of the analyzed MCL cells. Clinically, an aggressive course of disease including cerebral lymphoma involvement was noted in the reported patient. Hence, systematic studies addressing the incidence, biology and clinical behavior of this form of MCL seem to be justified in future.
authors.affiliation: Department of Hematology and Oncology, Georg-August-University, Göttingen, Germany.
authors.forename: Roland
authors.initials: R
authors.lastname: Schroers
authors.forename: York
authors.initials: Y
authors.lastname: Hildebrandt
authors.forename: Rainer
authors.initials: R
authors.lastname: Steffens
authors.forename: Sabrina
authors.initials: S
authors.lastname: Becker
authors.forename: Gudrun
authors.initials: G
authors.lastname: Ohms
authors.forename: Frederike
authors.initials: F
authors.lastname: von Bonin
authors.forename: Detlef
authors.initials: D
authors.lastname: Haase
authors.forename: Hans P
authors.initials: HP
authors.lastname: Bertsch
authors.forename: Lorenz
authors.initials: L
authors.lastname: Trümper
authors.forename: Frank
authors.initials: F
authors.lastname: Griesinger
mesh_terms: D015703:Antigens, CD / Q000097:blood* / Q000235:genetics*; D001402:B-Lymphocytes / Q000378:metabolism / Q000473:pathology; D002880:Chromosomes, Human, Pair 11 / Q000235:genetics; D002883:Chromosomes, Human, Pair 14 / Q000235:genetics; D019938:Cyclin D1 / Q000096:biosynthesis / Q000235:genetics; D015973:Gene Expression Regulation, Leukemic; D053804:Granzymes; D006801:Humans; D020522:Lymphoma, Mantle-Cell / Q000097:blood* / Q000150:complications / Q000235:genetics*; D008297:Male; D008562:Membrane Glycoproteins / Q000097:blood; D008875:Middle Aged; D009182:Mycosis Fungoides / Q000097:blood* / Q000150:complications / Q000235:genetics*; D054353:Perforin; D052899:Pore Forming Cytotoxic Proteins; D012697:Serine Endopeptidases / Q000097:blood; D014178:Translocation, Genetic; D014314:Trisomy / Q000235:genetics / Q000503:physiopathology
chemical_list: D015703:Antigens, CD; D008562:Membrane Glycoproteins; D052899:Pore Forming Cytotoxic Proteins; D054353:Perforin; D019938:Cyclin D1; D053804:Granzymes; D012697:Serine Endopeptidases; C509212:GZMA protein, human