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An Unusual Case of Hepatosplenic αβ T-Cell Lymphoma Presenting with Coombs’-Negative Hemolytic Anemia

Hepatosplenic T-cell lymphoma (HSTCL) is a rare and aggressive extranodal T-cell lymphoma that comprises <5% of peripheral T-cell lymphomas. The majority of cases harbor the γδ T-cell receptor (TCR), but recently, a few cases have been shown to express the αβ TCR. Comparison of these two subtypes...

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Autores principales: Ibrahim, Feryal A., Shanmugam, Vignesh, Amer, Aliaa, El-Omri, Halima, Al-Sabbagh, Ahmad, Taha, Ruba Y., Soliman, Dina S.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Libertas Academica 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4668954/
https://www.ncbi.nlm.nih.gov/pubmed/26688667
http://dx.doi.org/10.4137/CMO.S35120
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author Ibrahim, Feryal A.
Shanmugam, Vignesh
Amer, Aliaa
El-Omri, Halima
Al-Sabbagh, Ahmad
Taha, Ruba Y.
Soliman, Dina S.
author_facet Ibrahim, Feryal A.
Shanmugam, Vignesh
Amer, Aliaa
El-Omri, Halima
Al-Sabbagh, Ahmad
Taha, Ruba Y.
Soliman, Dina S.
author_sort Ibrahim, Feryal A.
collection PubMed
description Hepatosplenic T-cell lymphoma (HSTCL) is a rare and aggressive extranodal T-cell lymphoma that comprises <5% of peripheral T-cell lymphomas. The majority of cases harbor the γδ T-cell receptor (TCR), but recently, a few cases have been shown to express the αβ TCR. Comparison of these two subtypes (αβ and γδ) shows similar clinicopathologic and cytogenetic features; however, due to the paucity of reported cases, it is not clear whether they are prognostically distinct entities. We report a case of αβ HSTCL with a rather unusual presentation of Coombs’-negative hemolytic anemia. Diagnosis proved challenging due to an unusual blastoid morphology with the absence of typical intrasinusoidal distribution of tumor cells in the bone marrow. This unique case adds to the growing list of this rare subtype of T-cell lymphomas, which warrant urgent attention due to the lack of effective treatment options and dismal prognosis.
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spelling pubmed-46689542015-12-19 An Unusual Case of Hepatosplenic αβ T-Cell Lymphoma Presenting with Coombs’-Negative Hemolytic Anemia Ibrahim, Feryal A. Shanmugam, Vignesh Amer, Aliaa El-Omri, Halima Al-Sabbagh, Ahmad Taha, Ruba Y. Soliman, Dina S. Clin Med Insights Oncol Case Report Hepatosplenic T-cell lymphoma (HSTCL) is a rare and aggressive extranodal T-cell lymphoma that comprises <5% of peripheral T-cell lymphomas. The majority of cases harbor the γδ T-cell receptor (TCR), but recently, a few cases have been shown to express the αβ TCR. Comparison of these two subtypes (αβ and γδ) shows similar clinicopathologic and cytogenetic features; however, due to the paucity of reported cases, it is not clear whether they are prognostically distinct entities. We report a case of αβ HSTCL with a rather unusual presentation of Coombs’-negative hemolytic anemia. Diagnosis proved challenging due to an unusual blastoid morphology with the absence of typical intrasinusoidal distribution of tumor cells in the bone marrow. This unique case adds to the growing list of this rare subtype of T-cell lymphomas, which warrant urgent attention due to the lack of effective treatment options and dismal prognosis. Libertas Academica 2015-12-02 /pmc/articles/PMC4668954/ /pubmed/26688667 http://dx.doi.org/10.4137/CMO.S35120 Text en © 2015 the author(s), publisher and licensee Libertas Academica Ltd. This is an open access article published under the Creative Commons CC-BY-NC 3.0 license.
spellingShingle Case Report
Ibrahim, Feryal A.
Shanmugam, Vignesh
Amer, Aliaa
El-Omri, Halima
Al-Sabbagh, Ahmad
Taha, Ruba Y.
Soliman, Dina S.
An Unusual Case of Hepatosplenic αβ T-Cell Lymphoma Presenting with Coombs’-Negative Hemolytic Anemia
title An Unusual Case of Hepatosplenic αβ T-Cell Lymphoma Presenting with Coombs’-Negative Hemolytic Anemia
title_full An Unusual Case of Hepatosplenic αβ T-Cell Lymphoma Presenting with Coombs’-Negative Hemolytic Anemia
title_fullStr An Unusual Case of Hepatosplenic αβ T-Cell Lymphoma Presenting with Coombs’-Negative Hemolytic Anemia
title_full_unstemmed An Unusual Case of Hepatosplenic αβ T-Cell Lymphoma Presenting with Coombs’-Negative Hemolytic Anemia
title_short An Unusual Case of Hepatosplenic αβ T-Cell Lymphoma Presenting with Coombs’-Negative Hemolytic Anemia
title_sort unusual case of hepatosplenic αβ t-cell lymphoma presenting with coombs’-negative hemolytic anemia
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4668954/
https://www.ncbi.nlm.nih.gov/pubmed/26688667
http://dx.doi.org/10.4137/CMO.S35120
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