Hepatosplenic T-cell lymphoma, αβ type, presenting in an elderly patient status post indolent B-cell lymphoma treatment: report of a case and review of the literature

Hepatosplenic T-cell lymphoma (HSTL) is a rare and aggressive mature T-cell lymphoma characterized by hepatosplenic involvement. It usually occurs de novo in adolescents and young adults (AYA) and has been most commonly reported in the setting of immunosuppression. Most cases present with proliferation of cytotoxic T-cells of TCRγδ type with a unique gene mutation profile. Due to rarity of this disease, there is no standard of care treatment, and outcomes are usually poor in the absence of stem cell transplant. In this case, we report an unusual presentation of HSTL of TCRαβ type in an elderly patient status post treatment of an indolent B-cell lymphoma.

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Journal
Human Pathology Reports
Published
2025-08-22
DOI
https://doi.org/10.1016/j.hpr.2025.300789
Primary Topic
Lymphoma Diagnosis and Treatment
Type
article
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article

Hepatosplenic T-cell lymphoma, αβ type, presenting in an elderly patient status post indolent B-cell lymphoma treatment: report of a case and review of the literature

Isabela Chang, Jiehao Zhou, Allison Rosenthal, Rodolfo Garza-Morales
Human Pathology Reports
Lymphoma Diagnosis and Treatment
article

Hepatosplenic T-cell lymphoma, αβ type, presenting in an elderly patient status post indolent B-cell lymphoma treatment: report of a case and review of the literature

Isabela Chang, Jiehao Zhou, Allison Rosenthal, Rodolfo Garza-Morales
article en

Abstract

Hepatosplenic T-cell lymphoma (HSTL) is a rare and aggressive mature T-cell lymphoma characterized by hepatosplenic involvement. It usually occurs de novo in adolescents and young adults (AYA) and has been most commonly reported in the setting of immunosuppression. Most cases present with proliferation of cytotoxic T-cells of TCRγδ type with a unique gene mutation profile. Due to rarity of this disease, there is no standard of care treatment, and outcomes are usually poor in the absence of stem cell transplant. In this case, we report an unusual presentation of HSTL of TCRαβ type in an elderly patient status post treatment of an indolent B-cell lymphoma.

Human Pathology ReportsVol. 41
Mayo Clinic Hospital (US)
Good health and well-being
Openalex Percentile: Top 69%
Lymphoma Diagnosis and Treatment
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