SEER is an authoritative source of information on cancer incidence and survival in the United States. SEER currently collects and publishes cancer incidence and survival data from population-based cancer registries covering approximately 28 percent of the U.S. population.
An extranodal and systemic neoplasm derived from cytotoxic T-cells usually of Gamma Delta T-cell receptor type, medium in size, demonstrating marked sinusoidal infiltration of spleen, liver and bone marrow. Patients frequently have a history of chronic immunosuppression, including kidney transplantation.
Patients with hepatosplenic T-cell lymphoma present with marked splenomegaly, usually with hepatomegaly, but with no lymphadenopathy. Bone marrow is almost constantly involved.
The spleen is enlarged with diffuse involvement of the red pulp without any gross lesions. Diffuse hepatic enlargement is present as well. Patients present with hepatosplenomegaly and systemic symptoms, usually marked thrombocytopenia, often anemia and leukopenia. Peripheral blood involvement is uncommon at presentation but may occur later in the clinical course.
The disease course is aggressive. Patients may respond initially to chemotherapy but relapses are seen in the vast majority of cases. The median survival is <2 years. Removal of the spleen and stem cell transplants have been used in treatment of this disease.
Definitive Diagnostic Methods
2-5 copies of i(7)(q10)
Isochromosome 7q present
Numerical and structural aberrations of the second chromosome 7
Rearrangement TRG@, TRB@ genes
T-cell receptor (TCR) Gamma/Delta genes are clonally rearranged
Negative: CD4, CD5, granzyme B (Beta), perforin, CD94 (dim or absent), TCR Alpha Beta Positive/Negative: CD56, CD8TCRa