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

Yea Bing ThamAsral Wirda Ahmad AsnawiNgee Siang LauAlina Md FauziSharifah Shahnaz Syed Abd KadirPek Kuen LiewSen Mui Tan2024-06-162024-06-1620232024-1-29Yea 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/10.1159/0005319241662-6575597-3410.1159/000531924https://oarep.usim.edu.my/handle/123456789/19666Case Report in Oncology Volume 16 Issue 1 Page (827-836)Hepatosplenic 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 literatureen-USLymphoma · T cell · Cytopenia · Hepatomegaly · SplenomegalyCase Series of Hepatosplenic T-Cell Lymphoma: A Rare and Aggressive Diseasetext::journal::journal article821830162023