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Hepatosplenic T-cell lymphoma is a rare form of lymphoma that is generally incurable, except in the case of an allogeneic stem cell transplant. [ 2 ] [ 3 ] It is a systemic neoplasm comprising medium-sized cytotoxic T-cells that show significant sinusoidal infiltration in the liver , spleen , and bone marrow .
The human T-cell leukemia virus-1 is endemic in regions such as Japan and the Caribbean and has been associated with the increased risk of T-cell lymphoma such as Adult T-cell leukemia/lymphoma (ATL). [10] HTLV-1 has been attributed to 56% and 78% of all ATL cases in Japan and the Caribbean respectively. [9]
The use of rituximab has been established for the treatment of B-cell–derived hematologic malignancies, including follicular lymphoma (FL) and diffuse large B-cell lymphoma (DLBCL). [ 7 ] In addition to cure-directed treatment, people can benefit from self-care to manage symptoms.
Hepatosplenic T-cell lymphoma: Hepatosplenic T-cell lymphoma is a systemic neoplasm comprising medium-sized cytotoxic T-cells that show a significant sinusoidal infiltration in the liver, spleen, and bone marrow. [70] It is a rare and generally incurable form of lymphoma. [71] B-cell lymphoma: reticuloendotheliosis: reticulosis: Microglioma
Angioimmunoblastic T-cell lymphoma (AITL) is a fast-growing form of mature T-cell lymphoma, accounting for 18.5% of patients. [9] It is characterised by systemic disorders, polymorphous lymphoid infiltrate and a significant increase in proliferation of follicular dendritic cells and high endothelial venules. [10]
Adult T-cell leukemia/lymphoma (ATL or ATLL) is a rare cancer of the immune system's T-cells [1] [2] [3] caused by human T cell leukemia/lymphotropic virus type 1 . [4] All ATL cells contain integrated HTLV-1 provirus further supporting that causal role of the virus in the cause of the neoplasm. [ 4 ]
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