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.
(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010, see code 9680/3.)
Lymph nodes are usually spared. Two major patterns of clinical presentation have been recognized:
1. Western form characterized by symptoms related to the main organinvolved, predominantly neurological or cutaneous
2. Asian variant in which the patients present with multi-organ failure, hepatosplenomegaly, pancytopenia, and hemophagocytic syndrome. B symptoms very common in both types of presentations. This is an aggressive lymphoma which responds poorly to chemotherapy. The poor prognosis reflects in part frequent delays in diagnosis because of a lack of detectable tumormasses.
An isolated cutaneous variant has been identified invariably in Western females. It is characterized by limitation of tumor to the skin and has a better prognosis.