Splenic Marginal Zone Lymphoma

spleen from grey's anatomy
"Grey's Anatomy"-spleen highlighted in purple

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.

Diagnosis and Staging

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.

Diagnosis may involve any of the following:

  • Biopsy
  • Chest X-ray
  • PET scan
  • CT scan
  • Ultrasound
  • Bone marrow biopsy

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.

Treatment

There are various treatment options for SMZL:

Splenectomy
Surgical removal of the spleen. This can lead to full remission in some patients.
Immunotherapy
Rituxan is the most common form of treatment used for SMZL and is very effective. It is occasionally used in combination with chemotherapy.
Radiation
Irradiation of the spleen is sometimes necessary.
Clinical Trials
Cancer Patients have more options through clinical studies. Follow this link to learn more and find a clinical study opportunity near you.
Radioimmunotherapy
Zevalin shows promising results as a treatment for marginal zone lymphomas in clinical trials

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Non-Hodgkin's Lymphoma Information Pages:

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