Splenic Marginal Zone Lymphoma (SMZL) is a cancer of the B-cells found in the spleen. The spleen helps the body’s immune system by removing old red blood cells and producing antibodies. This disease is very rare, accounting for less than 1% of all Non-Hodgkin’s Lymphomas, and is rarely found in children. Women are slightly more susceptible than men.
This disease is classified as a marginal zone cancer because the mutation occurs in the marginal zone, or outer compartment, of the affected B-cells.
Patients usually have an enlarged spleen, which may cause discomfort or pain. Symptoms can be slow to develop. Patients may also have swollen, painless lymph nodes (though this is very rare) and experience fatigue.
Staging, as with other lymphomas, depends on the spread of the disease. It is common for patients to have cancer in both the spleen and bone marrow, though progression into the lymph nodes themselves is rare and marks the advanced stages of the disease.