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Grey Zone Lymphoma

Though rare, a lymphoma can have features of both classical Hodgkin lymphoma and diffuse large B-cell lymphoma (DLBCL).

The DLBCL subtype most commonly identified is ‘primary mediastinal B-cell lymphoma (PMBL). When the lymphoma has features of both types of lymphoma, it is sometimes referred to as ‘grey zone lymphoma’. It might also be called ‘B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma.’

Grey zone lymphoma can occur at any age but usually affects people aged 20–40. Men are affected more often than women.

Grey zone lymphoma usually starts in an area called the mediastinum, which is in the centre of the chest. Most people with grey zone lymphoma develop a large mass of lymphoma in this area. The mass can press on surrounding organs like the lungs. This can cause symptoms like breathing problems. The lymphoma can start in other areas, too.

How are rare high-grade B-cell lymphomas treated?

There is no standard treatment for grey zone lymphoma, double-hit or triple-hit lymphoma or high-grade B-cell lymphoma, NOS. Because these lymphomas are rare, it may take time to find out what treatment will work best.

Most people with grey zone lymphoma or high-grade B-cell lymphoma NOS have the same treatment used for DLBCL or PMBL. If your doctor thinks your lymphoma is more likely to behave like Burkitt lymphoma or classical Hodgkin lymphoma, instead, they might suggest the treatments commonly used for these types of lymphoma. You might be offered an intensive treatment if your lymphoma has features that suggest it may be difficult to treat.

Double-hit and triple-hit lymphoma can be difficult to treat and have higher relapse rates (the lymphoma coming back) than more common types of DLBCL. If you are fit enough, your doctor might suggest intensive chemo-immunotherapy (chemotherapy with antibody therapy). Some people might be offered a stem cell transplant after successful treatment. Stem cell transplants may reduce the risk of relapse.

Some people might be offered treatment as part of a clinical trial. Some clinical trials aim to find out which existing treatment regimen (combination of drugs) works best for these rare lymphomas. Other clinical trials test newer drugs and new combinations of treatments to find out if they are effective.

How can I find information about a rare high-grade B-cell lymphoma?

It can be difficult to find relevant information if your lymphoma does not fit into any of the more usual types. Ask your doctor for advice on what information is most relevant for you. They can advise you based on your individual circumstances.

Download the Grey Zone Lymphoma Fact Sheet.