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Splenic Marginal Zone Lymphoma (SMZL)

SMZL is a type of indolent B-cell NHL that predominantly involves the spleen. It is a rare type of lymphoma accounting for less than 1% of all NHL. It most commonly occurs in adults and slightly more frequently in women than in men.

What are the symptoms?

Symptoms do not normally appear until years after the disease has begun. The most common symptom is an enlarged spleen (splenomegaly). Unlike many other types of NHL, there are normally no swollen lymph nodes.

There is usually involvement of the blood and bone marrow at the time of diagnosis, with the only other symptom being fatigue.

Diagnosis is based on identification of the cell type together with the typical clinical findings. A biopsy of the bone marrow can often confirm diagnosis however removal of the spleen (splenectomy) is occasionally required for tissue examination.

How is it treated?

A number of different approaches may be taken with SMZL, including Watch and Wait, removal of the spleen (splenectomy), radiotherapy, chemotherapy medicines with a monoclonal antibody and targeted therapies.