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Hepatosplenic T-cell Lymphoma : Treatment

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Hepatosplenic T-cell lymphoma (HSTCL) is a highly aggressive disease with no effective treatment options. A variety of approaches have been tried, including combination chemotherapy regimens and autologous or allogeneic hematopoietic stem cell transplantation. However, none of these have produced durable remissions in the vast majority of cases.

With CHOP-like regimens as the first line of treatment, 60% to 70% of patients respond initially but experience early relapses. The median survival is about 16 months. The relapses occur in the sites of initial involvement. In rare cases, other sites such as skin, mucosa, or meninges may be involved. Many cases evolve into a lymphoma with large blast-like or highly pleomorphic cells (as shown here). Rare patients develop a frank leukemic phase.

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