Case of a CD3 Negative Hepatosplenic T-Cell Lymphoma: Diagnostic and Therapeutic Challenges

Hepatosplenic T-cell lymphomas (TCLs) are a rare, aggressive subset of TCLs, accounting for less than 5% of all peripheral T-cell and natural killer (NK) cell lymphomas. We report the case of a CD3 negative hepatosplenic T-cell lymphoma in a 42-year-old female, who presented with left-sided abdomina...

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Main Authors: Laura Alder, Scott Graupner, Guanhua Lai, Victor Yazbeck
Format: Article
Language:English
Published: Wiley 2019-01-01
Series:Case Reports in Hematology
Online Access:http://dx.doi.org/10.1155/2019/5315086
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author Laura Alder
Scott Graupner
Guanhua Lai
Victor Yazbeck
author_facet Laura Alder
Scott Graupner
Guanhua Lai
Victor Yazbeck
author_sort Laura Alder
collection DOAJ
description Hepatosplenic T-cell lymphomas (TCLs) are a rare, aggressive subset of TCLs, accounting for less than 5% of all peripheral T-cell and natural killer (NK) cell lymphomas. We report the case of a CD3 negative hepatosplenic T-cell lymphoma in a 42-year-old female, who presented with left-sided abdominal pain. She underwent a liver biopsy that showed marked abnormal sinusoidal lymphoid infiltration. PET scan revealed increased splenic and pharyngeal lymph node uptake. Immunophenotype was remarkable for negative CD3, gamma delta T-cell receptor, and alpha beta-T-cell receptor expression. She received 6 cycles of DA-EPOCH, had primary refractory disease and then underwent palliative splenectomy secondary to painful necrosis. Then, she was started on pralatrexate as a single agent and then in combination with romidepsin as a potential bridge to an allogeneic stem cell transplantation from her sibling.
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institution Kabale University
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spelling doaj-art-9fcecf4463a7468aa38938e73712752d2025-02-03T06:14:13ZengWileyCase Reports in Hematology2090-65602090-65792019-01-01201910.1155/2019/53150865315086Case of a CD3 Negative Hepatosplenic T-Cell Lymphoma: Diagnostic and Therapeutic ChallengesLaura Alder0Scott Graupner1Guanhua Lai2Victor Yazbeck3Department of Medicine, VCU Health, 1201 E Marshall St, Richmond, VA 23298, USADepartment of Hematology Oncology, Massey Cancer Center, VCU Health, 401 College St, Richmond, VA 23298, USADepartment of Pathology, VCU Health, 1201 E Marshall St, Richmond, VA 23298, USADepartment of Hematology Oncology, Massey Cancer Center, VCU Health, 401 College St, Richmond, VA 23298, USAHepatosplenic T-cell lymphomas (TCLs) are a rare, aggressive subset of TCLs, accounting for less than 5% of all peripheral T-cell and natural killer (NK) cell lymphomas. We report the case of a CD3 negative hepatosplenic T-cell lymphoma in a 42-year-old female, who presented with left-sided abdominal pain. She underwent a liver biopsy that showed marked abnormal sinusoidal lymphoid infiltration. PET scan revealed increased splenic and pharyngeal lymph node uptake. Immunophenotype was remarkable for negative CD3, gamma delta T-cell receptor, and alpha beta-T-cell receptor expression. She received 6 cycles of DA-EPOCH, had primary refractory disease and then underwent palliative splenectomy secondary to painful necrosis. Then, she was started on pralatrexate as a single agent and then in combination with romidepsin as a potential bridge to an allogeneic stem cell transplantation from her sibling.http://dx.doi.org/10.1155/2019/5315086
spellingShingle Laura Alder
Scott Graupner
Guanhua Lai
Victor Yazbeck
Case of a CD3 Negative Hepatosplenic T-Cell Lymphoma: Diagnostic and Therapeutic Challenges
Case Reports in Hematology
title Case of a CD3 Negative Hepatosplenic T-Cell Lymphoma: Diagnostic and Therapeutic Challenges
title_full Case of a CD3 Negative Hepatosplenic T-Cell Lymphoma: Diagnostic and Therapeutic Challenges
title_fullStr Case of a CD3 Negative Hepatosplenic T-Cell Lymphoma: Diagnostic and Therapeutic Challenges
title_full_unstemmed Case of a CD3 Negative Hepatosplenic T-Cell Lymphoma: Diagnostic and Therapeutic Challenges
title_short Case of a CD3 Negative Hepatosplenic T-Cell Lymphoma: Diagnostic and Therapeutic Challenges
title_sort case of a cd3 negative hepatosplenic t cell lymphoma diagnostic and therapeutic challenges
url http://dx.doi.org/10.1155/2019/5315086
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