Gray zone lymphoma is an aggressive and rare type of lymphoma. Because it’s rare, diagnosis and treatment can be difficult. However, people in the early stages respond well to chemotherapy, radiation, and stem cell transplants.

Gray zone lymphoma is a rare type of lymphoma that shares characteristics of both Hodgkin’s lymphoma and a type of non-Hodgkin’s lymphoma called primary mediastinal B-cell lymphoma (PMBCL).

Some people are treated for Hodgkin’s lymphoma and PMBCL unsuccessfully before they’re diagnosed with gray zone lymphoma. This confusion is what earned gray zone lymphoma its name when it was first recognized as a distinct type of lymphoma in 2008.

In this article, we’ll take a closer look at gray zone lymphoma, including its symptoms, diagnosis, treatment, and outlook.

Gray zone lymphoma causes swelling in your thymus, a small gland under your breastbone, and in the nearby lymph nodes. As the tumor grows and swelling increases, many people with gray zone lymphoma notice a lump in their chest, near their breast bone.

Sometimes, this swelling is painful or results in pressure in the chest. The tumor growth can also press on the lungs and throat, leading to difficulty eating, talking, and breathing.

Gray zone lymphoma also causes generalized lymphoma symptoms. These include:

Gray zone lymphoma is rare, and there’s no known cause for this cancer. Experts believe that infection with the Epstein-Barr virus can increase the risk of gray zone lymphoma. However, the virus doesn’t directly cause cancer, and people who’ve never had Epstein-Barr can develop gray zone lymphoma.

Research also shows that, while gray zone lymphoma can occur in people of any age or gender, it’s more common in people who are between 20 and 40 years old and in people assigned male at birth.

You’re also more at risk for gray zone lymphoma if you have any risk factors for all types of lymphoma:

  • having a family member with lymphoma
  • having a family member with chronic leukemia
  • having a condition that weakens your immune system
  • having an autoimmune condition
  • taking a medication that weakens your immune system
  • having prolonged workplace exposure to pesticides, herbicides, fungicides, solvents, paints, oil, fuel, infectious organisms, dust, or hair dye.
  • having any type of cancer in the past

The first step to getting diagnosed with gray zone lymphoma is a medical appointment. At your appointment, your doctor will ask you about your symptoms and your medical history. If they suspect you might have any type of lymphoma, they’ll order tests to help confirm the diagnosis.

Common tests for gray zone lymphoma include:

  • Blood tests: Blood tests will look at the numbers of red blood cells, white blood cells, and platelets in your blood. Lymphoma can affect how many of these cells are circulating in your blood.
  • Biopsies: Biopsies will be done on any swollen lymph nodes or glands. Biopsies remove samples of the affected node or gland so that it can be tested for cancer in a lab. Typically, biopsies for gray zone lymphoma are done by inserting a thin and hollow needle into the node or gland.

Biopsies are typically the best way to confirm cancer. Once you’ve been diagnosed with gray zone lymphoma, you’ll have additional tests to stage your cancer.

Staging is a system that addresses how far cancer has progressed. It helps doctors plan your treatment and helps you get a better understanding of your outlook. Gray zone lymphoma staging is based on:

  • how many areas of your body have lymphoma
  • which parts of your body have lymphoma
  • whether the lymphoma has spread to your bone marrow
  • whether the lymphoma has spread to organs, including the liver, skin, and lungs

Staging of gray zone lymphoma

There are four stages of gray zone lymphoma. In stages 1 and 2, the cancer is early stage and hasn’t yet spread throughout the body. In stages 3 and 4, cancer is advanced and has spread throughout the body, including to organs and bone marrow. The exact breakdown of stages is:

  • Stage 1: Cancer is contained to one lymph node.
  • Stage 2: Cancer is contained to two or more lymph nodes on the same side of the diaphragm
  • Stage 3: Cancer is in at least one lymph node above the diaphragm and one lymph node below the diaphragm.
  • Stage 4: Cancer has spread throughout the body and to distant organs.
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Gray zone lymphoma is rare, and there’s no standard treatment. Your doctor will look at your test results and develop the treatment plan that is best for you.

Common gray zone lymphoma treatments include:

Gray zone lymphoma is a rare and unique type of lymphoma that has only recently been recognized. This makes it difficult to obtain the kind of survival statistics that have been gathered for other cancers and conditions.

A 2020 study looked at accumulated data on patients with gray zone lymphoma across the United States. They found that people with gray zone lymphoma had a 68% chance of survival in the 3 years following their diagnosis.

The study also found that age at diagnosis and stage of cancer at diagnosis had a large impact on survival. Younger people who were treated in earlier stages saw much better outcomes.

Gray zone lymphoma is a rare type of lymphoma that is aggressive and often difficult to treat. It causes abnormal white blood cell growth and spread, along with swelling near the breastbone. This can lead to symptoms such as shortness of breath, chest pain, and difficulty eating, in addition to other standard symptoms of lymphoma.

Since gray zone lymphoma is rare, there isn’t a set treatment. However, many people respond well to a combination of chemotherapy and radiation therapy. Stem cell transplants are also a possibility.

The outlook for gray zone lymphoma can be difficult to predict, but younger people who receive treatment early often have the most successful outcomes.