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.’
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
How are rare high-grade B-cell
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.
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.
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.
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.
can I find information about a rare high-grade B-cell lymphoma?
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
Download the Grey Zone Lymphoma Fact Sheet.