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.
Sezary syndrome is a generalized mature T-cell lymphoma characterized by the presence of erythroderma, lymphadenopathy and neoplastic T-lymphocytes in the blood. The neoplastic T-cells have cerebriform nuclei, and the disease is by tradition regarded as a variant of Mycosis Fungoides. However, the behavior is usually much more aggressive.
Sezary syndrome (SS) is defined by the triad of erythroderma, generalized lymphadenopathy, and the presence of clonally related neoplastic T-cells with cerebriform nuclei (Sezary cells) in skin, lymph nodes, and peripheral blood. The histologic features in SS may be similar to those in Mycosis Fungoides; however, the cellular infiltrates in SS are more often monotonous, and epidermotropism may sometimes be absent.
SS is a leukemia and thus, by definition, a generalized disease. All visceral organs may be involved in the terminal stages, however, there is often a remarkable sparing of the bone marrow. This is an aggressive disease with an overall survival rate at 5 years of 10-20%. Most patients die of opportunistic infections. Prognostic factors include the degree of lymph node and peripheral blood involvement.