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Expression of MUM1/IRF4 selectively clusters with primary effusion lymphoma among lymphomatous effusions: implications for disease histogenesis and pathogenesis
Author(s) -
Carbone Antonino,
Gloghini Annunziata,
Cozzi Maria Rita,
Capello Daniela,
Steffan Agostino,
Monini Paolo,
De Marco Luigi,
Gaidano Gianluca
Publication year - 2000
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.2000.02329.x
Subject(s) - primary effusion lymphoma , biology , irf4 , lymphoma , germinal center , cancer research , serous fluid , pathology , histogenesis , b cell , immunology , immunohistochemistry , antibody , medicine , transcription factor , genetics , gene , biochemistry
Primary effusion lymphoma (PEL) is a peculiar B‐cell lymphoma characterized by infection by human herpesvirus type‐8/Kaposi sarcoma‐associated herpesvirus (HHV‐8/KSHV) and by preferential growth in the serous body cavities. Histogenetic studies have suggested that PEL originates from B cells at a late stage of differentiation. In this study, we have investigated PEL for the expression status of MUM1/IRF4 (multiple myeloma 1/interferon regulatory factor 4) protein, which is involved in physiological B‐cell maturation and represents a histogenetic marker of late B‐cell differentiation. Using multiple detection assays, all cases of PEL ( n = 22) were found to express MUM1/IRF4 molecules. MUM1/IRF4 expression was a selective feature of PEL among lymphomas involving the serous body cavities as secondary lymphomatous effusions generally failed to express the protein. In reactive lymphoid tissues, MUM1/IRF4 expression clustered with advanced stages of B‐cell differentiation. Comparison of MUM1/IRF4 expression with that of other histogenetic markers defined two phenotypic variants of PEL, i.e. MUM1/IRF4 + , CD138/syndecan‐1 + , B‐cell antigen − (20 out of 22 cases) and MUM1/IRF4 + , CD138/syndecan‐1 − , B‐cell antigen + (2 out of 22 cases), suggesting a certain degree of heterogeneity in the disease histogenesis. The implications of these data are threefold. First, MUM1/IRF4 expression corroborates the notion that PEL originates from post‐germinal centre, preterminally differentiated B‐cells. Second, MUM1/IRF4 may help in the differential diagnosis of PEL among other lymphomas involving the serous body cavities. Finally, MUM1/IRF4 may interact with HHV‐8/KSHV‐encoded interferon regulatory factors (IRFs) and thus contribute to PEL escape from interferon‐mediated control of viral infection.