<i>Bcl-6</i>and Lymphoproliferative Disorders

Gy, Michaud; Rd, Gascoyne; Bk, McNeil; Me, Anderson; Horsman, DE

doi:10.3109/10428199709050888

Cited by 6 publications

(4 citation statements)

References 31 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Increasing attention has focused on the potential oncogenic role of bcl ‐6 gene and Bcl‐6 protein alterations in NHL, especially diffuse large B‐cell lymphoma. The bcl ‐6 gene is located at chromosome 3q27, and structural changes at this locus, including translocations with immunoglobulin genes [t(3;14)(q27;q32) and t(3;22)(q27;q11)] have been described 40–42. The bcl ‐6 gene is rearranged in 30–40% of diffuse large B‐cell lymphomas and in 5–10% of follicular lymphomas 21–23, 28.…”

Section: Discussionmentioning

confidence: 99%

Primary follicular lymphoma of the testis in childhood

Finn

Viswanatha

Belasco

et al. 1999

Cancer

107

View full text Add to dashboard Cite

BACKGROUND Follicular lymphoma in childhood is rare. The authors present four unusual primary follicular lymphomas of the testis in children. METHODS Tumor tissue was evaluated using light microscopy, immunohistochemistry, flow cytometry, and polymerase chain reaction (PCR) for immunoglobulin heavy chain (IgH) and bcl‐2 gene rearrangements. Southern blot and immunohistochemical analyses were used to detect bcl‐6 gene rearrangements and protein expression, respectively. RESULTS Four young boys ranging in age from 3 to 10 years were diagnosed with Stage IE follicular large cell lymphoma (Grade 3). A B‐cell phenotype was documented in all four cases; monoclonality was confirmed in three cases by demonstration of light chain restriction or clonal IgH gene rearrangement. None of the lymphomas expressed Bcl‐2 or p53 protein, and bcl‐2 gene rearrangements were not found in the three lymphomas studied. In contrast, Bcl‐6 protein was expressed by all three lymphomas studied, and a bcl‐6 gene rearrangement was detected in the one case analyzed by Southern blot. All four boys were treated by orchiectomy and combination chemotherapy and are alive with no evidence of disease 18–44 months following their initial diagnoses. CONCLUSIONS Follicular lymphomas may rarely occur as primary testicular tumors in prepubertal boys and, when localized, appear to be associated with a favorable prognosis. In contrast to follicular lymphoma in adults, pediatric follicular lymphomas of the testis are usually of large cell type (Grade 3) and lack bcl‐2 or p53 abnormalities. The identification, in one case, of a bcl‐6 gene rearrangement suggests an alternate molecular pathogenesis for pediatric follicular lymphoma. Cancer 1999;85:1626–35. © 1999 American Cancer Society.

show abstract

Section: Discussionmentioning

confidence: 99%

Primary follicular lymphoma of the testis in childhood

Finn

Viswanatha

Belasco

et al. 1999

Cancer

107

View full text Add to dashboard Cite

show abstract

“…These translocations occur in the 5Ј flanking region of the gene leaving the coding sequence intact but with the regulatory sequences replaced by heterologous promoters, which leads to deregulated bcl-6 expression (36,42,52,71,72). Although most commonly associated with translocation to different loci in the immunoglobulin heavy-chain gene, the rearrangements are promiscuous with a variety of partners (41,42,73). Cases with 3q27 abnormalities do not necessarily demonstrate a bcl-6 rearrangement using Southern blot analysis and almost half of cases with molecular evidence of such a rearrangement in one study did not show cytogenetic evidence of a 3q27 abnormality (36,37,73).…”

Section: Discussionmentioning

confidence: 99%

“…Although most commonly associated with translocation to different loci in the immunoglobulin heavy-chain gene, the rearrangements are promiscuous with a variety of partners (41,42,73). Cases with 3q27 abnormalities do not necessarily demonstrate a bcl-6 rearrangement using Southern blot analysis and almost half of cases with molecular evidence of such a rearrangement in one study did not show cytogenetic evidence of a 3q27 abnormality (36,37,73). As in one FL in this study, occasional FL and DLBCL have rearrangements of both bcl-2 and bcl-6 (33,39,41,43).…”

Section: Discussionmentioning

confidence: 99%

Immunophenotypic and Genotypic Markers of Follicular Center Cell Neoplasia in Diffuse Large B-Cell Lymphomas

King¹,

Chen²,

Locker³

et al. 2000

Modern Pathology

View full text Add to dashboard Cite

Diffuse large B-cell lymphomas (DLBCL) are a biologically and clinically heterogeneous entity. Although some DLBCL represent transformation of follicular lymphomas (FL), the proportion that is of follicular center cell (FCC) origin remains uncertain. Immunophenotypic and genotypic markers used to suggest a FCC origin for a lymphoma (bcl-6 and CD10 expression, lack of CD138 expression, bcl-2 rearrangements [R]) or to subdivide DLBCL (bcl-2 expresssion, bcl-6 R) were therefore investigated in 22 FL and 44 DLBCL using paraffin section immunostains and Southern blot/polymerase chain reaction analysis. All FL tested were bcl-6؉ (19) and CD138؊ (22) with 16/19 also bcl-2 and CD10؉ (classic phenotype), one bcl2؉, CD10؊ (grade III) and two bcl2؊, CD10؉ (grade II or III). Bcl-2R was identified in 4/5 FL-GrI, 3/6 FL-GrII, and 1/3 FL-GrIII. Bcl-6R was found in 0/5, 2/4, and 0/3 FL, respectively. All but 3/41 DLBCL were bcl-6؉ with 17/37 also bcl-2؉ and CD10؉. Three of these cases were also CD138؉. Twelve bcl-6؉ cases were bcl-2؉, CD10؊, six bcl-2؊, CD10؉, and two bcl-2؊, CD10؊. The three bcl-6؊ cases were bcl-2؉, CD138؊ and two were CD10؉. Bcl-2R was identified in 5/27 DLBCL with 4/5 bcl-2؉, 3/4 tested CD10؉ and 4/4 bcl-6؉. Bcl-6R was identified in 7/26 including three with a classic FL phenotype. The vast majority of DLBCL in this study have an immunophenotype that supports a FCC origin. Although the proportion of DLBCL that co-expressed bcl-6, CD10 and bcl-2 was lower than for the FL, absence of bcl-2 or CD10 may be associated with higher grade FL. It is also possible that bcl-6 expression is not completely specific for a FCC origin. Only a minority of cases suggested postfollicular differentiation. Only a minority of DLBCL show bcl-2R, suggesting that many have a different molecular pathogenesis than most low-grade FL. Bcl-6R did not exclude a FCC origin. KEY WORDS: BCL-2; BCL-6; CD10; CD138; diffuse large B-cell lymphoma; follicular lymphoma; germinal center Mod Pathol 2000;13(11):1219 -1231Diffuse large B-cell lymphomas (DLBCL), as defined in the REAL and upcoming WHO lymphoma classifications, represent a heterogeneous entity that includes not only transformed (noncleaved or centroblastic) follicular center cell (FCC) lymphomas but also all other lymphomas composed of large and transformed B cells (1-3). The proposed WHO classification recognizes three relatively uncommon subtypes of DLBCL [mediastinal (thymic), intravascular, and primary effusion lymphoma] but the immunoblastic lymphomas, transformed/"high grade" marginal zone lymphomas of mucosaassociated lymphoid tissue (MALT) and presumably nodal types and all other types of transformed B-cell lymphomas are not distinguished from one another. The DLBCL were grouped together for two main reasons. First, it was difficult to reproduce some of the previously described morphologic distinctions (such as those between transformed FCC lymphomas and immunoblastic lymphomas). Second, it was unclear how, for example, large noncleaved (LNC) FCC/centroblastic lymphomas should ...

show abstract

“…This suggests that another gene, probably located on 3q21, is responsible for increased thrombopoiesis and/or leukemic transformation. The CALLA antigen gene and the BCL-6 gene have also been mapped to this region [44,45], and BCL-6 rearrangements have been reported at 3q27 in patients with lymphomas [46,47].…”

Section: Discussionmentioning

confidence: 99%