What is Bing Neel Syndrome (BNS)?

What is it?

BNS is a rare complication of Waldenstrom’s macroglobulinaemia, where the abnormal cells your body is producing enter your spinal cord or brain. This causes problems in the central nervous system, that may progressively get worse as time goes by.

BNS can develop at any time during someone’s WM journey: before they receive a diagnosis, at the same time as their diagnosis or when they are in remission following treatment. You may not have WM symptoms alongside your BNS ones, as sometimes BNS can develop even when your WM is in remission.

How is it diagnosed?

There isn’t one test that can give a certain diagnosis of BNS. Instead, if you doctor suspects that you may have BNS, they will order a series of tests to determine the cause. You doctor should consider referring you to a centre that has expertise in treating BNS as it’s helpful to have input from haematologists and neurologists who are familiar with this rare condition and treat it regularly.

Your healthcare team will decide which tests you need, depending on your individual circumstances. The following may be performed:

A lumbar puncture, where a small sample of fluid is taken from your spinal cord through a thin needle to check if WM cells are there. This is done under local anaesthetic and shouldn’t be painful, although you may have backache or headache for a few days afterwards

Less common is a brain biopsy. This is where a sample of tissue from your brain is taken to see if WM cells are in your central nervous system. This is performed under general anaesthetic by a neurosurgeon

The following tests are also often necessary to support the diagnosis:

An MRI scan of the head and spinal cord may help doctors see if there is any sign of BNS in your body

Blood tests can help doctors understand more about your Waldenstrom’s macroglobulinaemia.

How can it be treated?

BNS is a rare condition, affecting around 1% of people living with Waldenstrom’s macroglobulinaemia. Therefore, it is important that if diagnosed you are treated in a hospital where healthcare professionals regularly treat people with the disease. If in doubt, speak to your doctor about getting a second opinion.

​There is no standard treatment for BNS, and any decision about treatment will depend on your individual circumstances, like your symptoms, fitness and any previous treatment.

Some of the treatments for BNS may be familiar to you, as they are similar to those used to treat WM. Other treatments are similar to those used to treat other diseases of the central nervous system. These include:

Steroids – hormones used to kill WM cells. However this treatment has been shown to only work in the short term and your BNS could come back quickly after finishing the treatment

Chemotherapy including methotrexate, cytarabine and rituximab. The chemotherapy combination MATRix (methotrexate, cytarabine, rituximab and thiotepa) has been used to treat high grade central nervous system lymphoma

Ibrutinib – a drug known as a BTK inhibitor, which has shown to be effective in treating people with BNS. This drug is available for people living in Scotland, but it no longer available for new patients in England and Wales.

Stem cell transplant – an intense therapy that isn’t suitable for everyone and is only considered in patients who are fit

Radiotherapy – which targets your brain with radiation to kill cells. This is only recommended in special circumstances, usually if other treatment is not suitable

Rituximab and bendamustine – a chemo-immunotherapy often used as a first line treatment for WM. Rituximab alone is not advised for the treatment of BNS