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Hum Pathol ; 125: 48-58, 2022 07.
Artículo en Inglés | MEDLINE | ID: mdl-35452696

RESUMEN

Normal T cells express high levels of B-cell lymphoma-2 (BCL2) protein, and data regarding BCL2 expression status and its diagnostic utility in T-cell lymphoma are scarce. We evaluated BCL2 expression in a series of mature T-cell lymphoproliferations (TCLs) including indolent and more recently recognized entities (follicular helper T-cell [TFH] lymphomas). Sixty-six neoplastic biopsies (60 patients) representing mature nodal, extranodal, and leukemia T-cell neoplasms were collected from three institutes (2 US and 1 Japan) and were compared with reactive T cells in 8 benign tissues/blood and 9 T cell-rich B-cell proliferations. BCL2 immunostaining was performed and scored based on intensity-weighted H-score (0-300). Next-generation sequencing (NGS; 5 cases), BCL2 gene sequencing, and real-time polymerase chain reaction (PCR; 3 cases) were conducted. Association of H-score with overall survival (using proportional hazards modeling) was assessed in nonleukemic TCLs. Most TCLs showed significantly downregulated median BCL2 H-score (125, range: 18-300) with the exception of T-cell prolymphocytic leukemia and hepatosplenic T-cell lymphoma, both of which showed uniform strong retention of BCL2 as did the 8 reactive tissues (median H-score: 280; p = 0.000). Notably all TFH lymphoma CD4 neoplastic T cells, subcutaneous panniculitis-like T-cell lymphoma, CD8 adipocyte-rimming T cells, and T-cell large lymphocyte leukemia with pathogenic STAT5B and TP53 mutation showed BCL2 downregulation. No BCL2 mutations were observed by NGS or sequencing with decreased BCL2 mRNA transcripts by real-time PCR. BCL2 downregulation is pervasive among many TCLs and unrelated to any mutations. There is utility for BCL2 immunostaining in some challenging situations as discussed in this article.


Asunto(s)
Leucemia , Linfoma de Células B , Linfoma de Células T Periférico , Linfoma de Células T , Proteínas Proto-Oncogénicas c-bcl-2 , Regulación hacia Abajo , Humanos , Linfoma de Células B/genética , Linfoma de Células B/metabolismo , Linfoma de Células B/patología , Linfoma de Células T/genética , Linfoma de Células T/metabolismo , Linfoma de Células T/patología , Linfoma de Células T Periférico/metabolismo , Linfoma de Células T Periférico/patología , Fenotipo , Proteínas Proto-Oncogénicas c-bcl-2/genética , Proteínas Proto-Oncogénicas c-bcl-2/metabolismo
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