Primary cutaneous marginal zone B-cell lymphoma may exhibit both the t(14;18)(q32;q21) IGH/BCL2 and the t(14;18)(q32;q21) IGH/MALT1 translocation: an indicator for clonal transformation towards higher-grade B-cell lymphoma? 2007

Gabriele Palmedo, and Markus Hantschke, and Arno Rütten, and Thomas Mentzel, and Werner Kempf, and Dario Tomasini, and Heinz Kutzner
DermPath, Friedrichshafen, Germany. palmedo@dermpath.de

Primary cutaneous marginal zone B-cell lymphoma (PCMZL) is a recently proposed entity and constitutes the cutaneous counterpart of extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT). The t(14;18)(q32;q21) involving the IGH and the MALT1 gene has previously been described in PCMZL, whereas the t(14;18)(q32;q21) IGH/BCL2 seems to be restricted to follicular lymphoma and diffuse large B-cell lymphoma. We screened 30 PCMZLs of 13 patients by fluorescent in situ hybridization analysis for the presence of the t(14;18)(q32;q21) IGH/BCL2 and the t(14;18)(q32;q21)IGH/MALT1. The t(14;18)(q32;q21) IGH/MALT1 was detected in 10 PCMZLs of eight patients, with four patients showing the t(14;18)(q32;q21) IGH/MALT1 exclusively. The t(14;18)(q32;q21)IGH/BCL2 was detected in 16 PCMZLs of seven patients, with four patients showing the t(14;18)(q32;q21) IGH/BCL2 exclusively. Six lymphomas of four patients showed both translocations in the same lesion. In seven lymphomas, neither of the two translocations occurred. One patient developed multiple lesions without either of the two translocations. Our results underline that both the t(14;18)(q32;q21)IGH/BCL2 and the t(14;18)(q32;q21) IGH/MALT1 may occur in PCMZL, albeit in an irregular distribution. Therefore, the etiopathogenetic relevance of either translocation in PCMZL remains a matter of debate.

UI MeSH Term Description Entries
D009363 Neoplasm Proteins Proteins whose abnormal expression (gain or loss) are associated with the development, growth, or progression of NEOPLASMS. Some neoplasm proteins are tumor antigens (ANTIGENS, NEOPLASM), i.e. they induce an immune reaction to their tumor. Many neoplasm proteins have been characterized and are used as tumor markers (BIOMARKERS, TUMOR) when they are detectable in cells and body fluids as monitors for the presence or growth of tumors. Abnormal expression of ONCOGENE PROTEINS is involved in neoplastic transformation, whereas the loss of expression of TUMOR SUPPRESSOR PROTEINS is involved with the loss of growth control and progression of the neoplasm. Proteins, Neoplasm
D002471 Cell Transformation, Neoplastic Cell changes manifested by escape from control mechanisms, increased growth potential, alterations in the cell surface, karyotypic abnormalities, morphological and biochemical deviations from the norm, and other attributes conferring the ability to invade, metastasize, and kill. Neoplastic Transformation, Cell,Neoplastic Cell Transformation,Transformation, Neoplastic Cell,Tumorigenic Transformation,Cell Neoplastic Transformation,Cell Neoplastic Transformations,Cell Transformations, Neoplastic,Neoplastic Cell Transformations,Neoplastic Transformations, Cell,Transformation, Cell Neoplastic,Transformation, Tumorigenic,Transformations, Cell Neoplastic,Transformations, Neoplastic Cell,Transformations, Tumorigenic,Tumorigenic Transformations
D002883 Chromosomes, Human, Pair 14 A specific pair of GROUP D CHROMOSOMES of the human chromosome classification. Chromosome 14
D002887 Chromosomes, Human, Pair 18 A specific pair of GROUP E CHROMOSOMES of the human chromosome classification. Chromosome 18
D002999 Clone Cells A group of genetically identical cells all descended from a single common ancestral cell by mitosis in eukaryotes or by binary fission in prokaryotes. Clone cells also include populations of recombinant DNA molecules all carrying the same inserted sequence. (From King & Stansfield, Dictionary of Genetics, 4th ed) Clones,Cell, Clone,Cells, Clone,Clone,Clone Cell
D004273 DNA, Neoplasm DNA present in neoplastic tissue. Neoplasm DNA
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000074884 Mucosa-Associated Lymphoid Tissue Lymphoma Translocation 1 Protein A caspase-like cysteine endopeptidase that also exhibits ubiquitin ligase activity. It contains an N-terminal DEATH DOMAIN, two IMMUNOGLOBULIN-LIKE DOMAINS, and localizes to the perinuclear region of MONOCYTES, where it functions in activation of NF-KAPPA B; it also binds to and activates TRAF6. Chromosomal translocations involving the MALT1 and BIRC2 genes are associated with MALT LYMPHOMA, and mutations in the MALT1 gene are associated with Type 12 IMMUNODEFICIENCY SYNDROMES. MALT-1 Protein,MALT1 Paracaspase,MALT1 Protein,Paracaspase,MALT 1 Protein,Mucosa Associated Lymphoid Tissue Lymphoma Translocation 1 Protein,Paracaspase, MALT1
D012878 Skin Neoplasms Tumors or cancer of the SKIN. Cancer of Skin,Skin Cancer,Cancer of the Skin,Neoplasms, Skin,Cancer, Skin,Cancers, Skin,Neoplasm, Skin,Skin Cancers,Skin Neoplasm
D014178 Translocation, Genetic A type of chromosome aberration characterized by CHROMOSOME BREAKAGE and transfer of the broken-off portion to another location, often to a different chromosome. Chromosomal Translocation,Translocation, Chromosomal,Chromosomal Translocations,Genetic Translocation,Genetic Translocations,Translocations, Chromosomal,Translocations, Genetic

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