Hepatosplenic T-Cell Lymphoma: A Rare Complication of Monotherapy with Thiopurines in Crohn’s Disease

Hepatosplenic T-cell lymphoma (HSTCL) is an extremely rare and aggressive form of non-Hodgkin lymphoma associated with poor response to treatment and high mortality. There is an increased incidence among patients with inflammatory bowel disease, especially young male patients under 35 years old and...

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Main Authors: Joana Carvão, Vítor Magno Pereira, Fernando Jacinto, Carla Sousa Andrade, Luís Jasmins
Format: Article
Language:English
Published: Karger Publishers 2018-11-01
Series:GE: Portuguese Journal of Gastroenterology
Subjects:
Online Access:https://www.karger.com/Article/FullText/493350
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author Joana Carvão
Vítor Magno Pereira
Fernando Jacinto
Carla Sousa Andrade
Luís Jasmins
author_facet Joana Carvão
Vítor Magno Pereira
Fernando Jacinto
Carla Sousa Andrade
Luís Jasmins
author_sort Joana Carvão
collection DOAJ
description Hepatosplenic T-cell lymphoma (HSTCL) is an extremely rare and aggressive form of non-Hodgkin lymphoma associated with poor response to treatment and high mortality. There is an increased incidence among patients with inflammatory bowel disease, especially young male patients under 35 years old and on combination therapy (thiopurine and anti-TNF-α). We describe a case of HSTCL in a young male patient with stenosing ileal Crohn’s disease on azathioprine monotherapy for 4.8 years admitted to our hospital with intra- abdominal sepsis. Despite chemotherapy, the patient eventually died 1 month after the diagnosis. Through a literature review, we identified 18 additional cases of HSTCL in Crohn’s disease patients that had only been treated with thiopurine monotherapy. The authors intend to highlight the rarity of this diagnosis especially with azathioprine monotherapy and the diagnostic challenge in a case that presented with intra-abdominal sepsis.
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spelling doaj.art-54436726c48b487b8f5f19bc90452ccc2022-12-21T17:49:13ZengKarger PublishersGE: Portuguese Journal of Gastroenterology2341-45452387-19542018-11-011610.1159/000493350493350Hepatosplenic T-Cell Lymphoma: A Rare Complication of Monotherapy with Thiopurines in Crohn’s DiseaseJoana CarvãoVítor Magno PereiraFernando JacintoCarla Sousa AndradeLuís JasminsHepatosplenic T-cell lymphoma (HSTCL) is an extremely rare and aggressive form of non-Hodgkin lymphoma associated with poor response to treatment and high mortality. There is an increased incidence among patients with inflammatory bowel disease, especially young male patients under 35 years old and on combination therapy (thiopurine and anti-TNF-α). We describe a case of HSTCL in a young male patient with stenosing ileal Crohn’s disease on azathioprine monotherapy for 4.8 years admitted to our hospital with intra- abdominal sepsis. Despite chemotherapy, the patient eventually died 1 month after the diagnosis. Through a literature review, we identified 18 additional cases of HSTCL in Crohn’s disease patients that had only been treated with thiopurine monotherapy. The authors intend to highlight the rarity of this diagnosis especially with azathioprine monotherapy and the diagnostic challenge in a case that presented with intra-abdominal sepsis.https://www.karger.com/Article/FullText/493350Hepatosplenic T-cell lymphomaCrohn’s diseaseThiopurines
spellingShingle Joana Carvão
Vítor Magno Pereira
Fernando Jacinto
Carla Sousa Andrade
Luís Jasmins
Hepatosplenic T-Cell Lymphoma: A Rare Complication of Monotherapy with Thiopurines in Crohn’s Disease
GE: Portuguese Journal of Gastroenterology
Hepatosplenic T-cell lymphoma
Crohn’s disease
Thiopurines
title Hepatosplenic T-Cell Lymphoma: A Rare Complication of Monotherapy with Thiopurines in Crohn’s Disease
title_full Hepatosplenic T-Cell Lymphoma: A Rare Complication of Monotherapy with Thiopurines in Crohn’s Disease
title_fullStr Hepatosplenic T-Cell Lymphoma: A Rare Complication of Monotherapy with Thiopurines in Crohn’s Disease
title_full_unstemmed Hepatosplenic T-Cell Lymphoma: A Rare Complication of Monotherapy with Thiopurines in Crohn’s Disease
title_short Hepatosplenic T-Cell Lymphoma: A Rare Complication of Monotherapy with Thiopurines in Crohn’s Disease
title_sort hepatosplenic t cell lymphoma a rare complication of monotherapy with thiopurines in crohn s disease
topic Hepatosplenic T-cell lymphoma
Crohn’s disease
Thiopurines
url https://www.karger.com/Article/FullText/493350
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AT fernandojacinto hepatosplenictcelllymphomaararecomplicationofmonotherapywiththiopurinesincrohnsdisease
AT carlasousaandrade hepatosplenictcelllymphomaararecomplicationofmonotherapywiththiopurinesincrohnsdisease
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