Splenic Marginal Zone Lymphoma

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

Dive Deeper

Non-Hodgkin's Lymphoma Information Pages:

More Articles

More Articles

In non-Hodgkin's lymphoma, you have your B-cell lymphomas and you have your T-cell lymphomas.

Why B...

MALT lymphoma is a rare B-cell non-Hodgkin's lymphoma that typically runs an indolent or slow-growing clinical...

Mantle cell lymphoma (MCL) is a relatively rare B-cell subtype of non-Hodgkin'...

Mantle cell lymphoma (MCL) is just one of 50-60 known B-cell subtypes of...

Lymphomatous meningitis [LM], also known as leukemic meningitis, is an extremely serious peripheral cancer that attacks the tissue that covers the...

Since so many chemotherapy agents can affect a patient’s sex drive and fertility, thinking about these issues prior...

Secondary cancers are cancers that develop as a result of chemotherapy and/or...

One of the greatest fears of lymphoma survivors is that they’ll relapse and have to undergo treatment again. This fear is normal but awful to...

Over the years, various classification systems have been used to differentiate lymphoma types including the Rappaport Classification (used until...

If you are new to this website or are looking for guidance to a specific page, here is a list of links to articles that can help you. The "Main...

Often the one who makes the first diagnosis of Hodgkin's Lymphoma / Disease is the person affected. There are some...

Lymphedema is abnormal swelling due to the presence of excess lymphatic fluid within the tissues. This swelling occurs when the...

There are two types of cancer: benign and malignant. Benign cancers are the kind that don't spread and don't threaten one's life. Malignant...

Advances in the treatment of Hodgkin's lymphoma have resulted in remarkable survival rates, even for...