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Case Series of Hepatosplenic T-Cell Lymphoma: A Rare and Aggressive Disease

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dc.contributor.authorYea Bing Tham
dc.contributor.authorAsral Wirda Ahmad Asnawi
dc.contributor.authorNgee Siang Lau
dc.contributor.authorAlina Md Fauzi
dc.contributor.authorSharifah Shahnaz Syed Abd Kadir
dc.contributor.authorPek Kuen Liew
dc.contributor.authorSen Mui Tan
dc.date.accessioned2024-06-16T06:31:19Z
dc.date.available2024-06-16T06:31:19Z
dc.date.issued2023
dc.date.submitted2024-1-29
dc.descriptionCase Report in Oncology Volume 16 Issue 1 Page (827-836)
dc.description.abstractHepatosplenic T-cell lymphoma is a rare form of T-cell lymphoma that predominantly emerges from neoplastic proliferation of cytotoxic T cells of γ/δ T-cell receptor-expressing lymphocytes. Isochromosome 7q and trisomy 8 are the most prevalent chromosomal abnormalities associated with hepatosplenic T-cell lymphoma, and most patients have mutations in genes related to chromatin remodeling or the JAK/STAT system. Hepatosplenic T-cell lymphoma can mimic various infectious diseases, immunological conditions, and other malignancies. Patients usually present with nonspecific constitutional symptoms and spleen and liver enlargement, with variable degrees of cytopenia. The rarity of this disease, coupled with the lack of lymph node involvement that is usually seen in lymphomas, causes significant difficulty in diagnosis, which inevitably delays the initiation of treatment. Managing this lymphoma is arduous because of its late presentation and aggressive nature, frequently resulting in rapid progression in its clinical course and refractoriness to conventional chemotherapy. There is a lack of international guidelines for its treatment, and in most cases, treatment is guided by case series. Here, we highlight the clinicopathological features and management of hepatosplenic T-cell lymphoma over a 10-year span in a single hematology referral center and review the literature
dc.identifier.citationYea Bing Tham, Asral Wirda Ahmad Asnawi, Ngee Siang Laua Alina Md Fauzi, Sharifah Shahnaz Syed Abd Kadir, Pek Kuen Liewa Sen Mui Tan (2023). Case Series of Hepatosplenic T-Cell Lymphoma: A Rare and Aggressive Disease. Case Reports in Oncology, 16(1), 827–836. https://doi.org/10.1159/000531924
dc.identifier.doi10.1159/000531924
dc.identifier.epage830
dc.identifier.issn1662-6575
dc.identifier.issue2023
dc.identifier.other597-34
dc.identifier.spage821
dc.identifier.urihttps://oarep.usim.edu.my/handle/123456789/19666
dc.identifier.volume16
dc.language.isoen_US
dc.publisherKarger
dc.relation.ispartofCase Reports in Oncology
dc.relation.issn1662-6575
dc.relation.journalCase Report in Oncology
dc.subjectLymphoma · T cell · Cytopenia · Hepatomegaly · Splenomegaly
dc.titleCase Series of Hepatosplenic T-Cell Lymphoma: A Rare and Aggressive Disease
dc.typetext::journal::journal article
dspace.entity.typePublication
oaire.citation.endPage836
oaire.citation.issue1
oaire.citation.startPage827
oaire.citation.volume16
oairecerif.author.affiliation#PLACEHOLDER_PARENT_METADATA_VALUE#
oairecerif.author.affiliationUniversiti Sains Islam Malaysia
oairecerif.author.affiliation#PLACEHOLDER_PARENT_METADATA_VALUE#
oairecerif.author.affiliationUniversiti Sains Islam Malaysia
oairecerif.author.affiliation#PLACEHOLDER_PARENT_METADATA_VALUE#
oairecerif.author.affiliation#PLACEHOLDER_PARENT_METADATA_VALUE#
oairecerif.author.affiliation#PLACEHOLDER_PARENT_METADATA_VALUE#

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