Early progression and transformation of a splenic diffuse red pulp small B-cell lymphoma with NOTCH1, ARID2, CREBBP, and TNFRSF14 gene mutations

Splenic diffuse red pulp small B-cell lymphoma (SDRPL) is a rare entity. Diagnosis is typically achieved with splenectomy and most patients remain in remission after this intervention. Hemoglobin value less than 10 g/dL and NOTCH1, TP53, and MAP2K1 gene mutations at diagnosis have been associated wi...

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Autores principales: Lopedote, Paolo, Nawaiseh, Ahmad Al, Malek, Anita, Faller, Garrey, Hattar, Mariana, Dow, Edward, Kozyreva, Olga
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2023
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10471916/
https://www.ncbi.nlm.nih.gov/pubmed/37664441
http://dx.doi.org/10.1016/j.lrr.2023.100384
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author Lopedote, Paolo
Nawaiseh, Ahmad Al
Malek, Anita
Faller, Garrey
Hattar, Mariana
Dow, Edward
Kozyreva, Olga
author_facet Lopedote, Paolo
Nawaiseh, Ahmad Al
Malek, Anita
Faller, Garrey
Hattar, Mariana
Dow, Edward
Kozyreva, Olga
author_sort Lopedote, Paolo
collection PubMed
description Splenic diffuse red pulp small B-cell lymphoma (SDRPL) is a rare entity. Diagnosis is typically achieved with splenectomy and most patients remain in remission after this intervention. Hemoglobin value less than 10 g/dL and NOTCH1, TP53, and MAP2K1 gene mutations at diagnosis have been associated with worse outcome. Progression after splenectomy of SDRPL is possible, although transformation to aggressive lymphoma has rarely been reported. We herein report the case of a patient formerly diagnosed with SDRPL with gene mutations involving CREBBP, NOTCH1, ARID2, and TNFRSF14 who transformed to diffuse large B-cell lymphoma six months after splenectomy.
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spelling pubmed-104719162023-09-02 Early progression and transformation of a splenic diffuse red pulp small B-cell lymphoma with NOTCH1, ARID2, CREBBP, and TNFRSF14 gene mutations Lopedote, Paolo Nawaiseh, Ahmad Al Malek, Anita Faller, Garrey Hattar, Mariana Dow, Edward Kozyreva, Olga Leuk Res Rep Article Splenic diffuse red pulp small B-cell lymphoma (SDRPL) is a rare entity. Diagnosis is typically achieved with splenectomy and most patients remain in remission after this intervention. Hemoglobin value less than 10 g/dL and NOTCH1, TP53, and MAP2K1 gene mutations at diagnosis have been associated with worse outcome. Progression after splenectomy of SDRPL is possible, although transformation to aggressive lymphoma has rarely been reported. We herein report the case of a patient formerly diagnosed with SDRPL with gene mutations involving CREBBP, NOTCH1, ARID2, and TNFRSF14 who transformed to diffuse large B-cell lymphoma six months after splenectomy. Elsevier 2023-08-22 /pmc/articles/PMC10471916/ /pubmed/37664441 http://dx.doi.org/10.1016/j.lrr.2023.100384 Text en © 2023 The Authors. Published by Elsevier Ltd. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Article
Lopedote, Paolo
Nawaiseh, Ahmad Al
Malek, Anita
Faller, Garrey
Hattar, Mariana
Dow, Edward
Kozyreva, Olga
Early progression and transformation of a splenic diffuse red pulp small B-cell lymphoma with NOTCH1, ARID2, CREBBP, and TNFRSF14 gene mutations
title Early progression and transformation of a splenic diffuse red pulp small B-cell lymphoma with NOTCH1, ARID2, CREBBP, and TNFRSF14 gene mutations
title_full Early progression and transformation of a splenic diffuse red pulp small B-cell lymphoma with NOTCH1, ARID2, CREBBP, and TNFRSF14 gene mutations
title_fullStr Early progression and transformation of a splenic diffuse red pulp small B-cell lymphoma with NOTCH1, ARID2, CREBBP, and TNFRSF14 gene mutations
title_full_unstemmed Early progression and transformation of a splenic diffuse red pulp small B-cell lymphoma with NOTCH1, ARID2, CREBBP, and TNFRSF14 gene mutations
title_short Early progression and transformation of a splenic diffuse red pulp small B-cell lymphoma with NOTCH1, ARID2, CREBBP, and TNFRSF14 gene mutations
title_sort early progression and transformation of a splenic diffuse red pulp small b-cell lymphoma with notch1, arid2, crebbp, and tnfrsf14 gene mutations
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10471916/
https://www.ncbi.nlm.nih.gov/pubmed/37664441
http://dx.doi.org/10.1016/j.lrr.2023.100384
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