Hepatosplenic T-cell lymphoma

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A rare T-cell non-Hodgkin lymphoma characterized by a proliferation of cytotoxic T-cells usually gamma delta T-cells with involvement of the liver and spleen but without involvement of lymph nodes. The bone marrow is consistently affected. Patients typically present during adolescence or young adulthood with hepatosplenomegaly pancytopenia and systemic symptoms. Peripheral blood involvement may develop later in the disease course. There is a clear male preponderance. The disease often occurs in the context of long-term immunosuppression. The course is aggressive with poor therapy response.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version June 2024

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Hepatosplenic T-cell lymphoma?

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