Newly Diagnosed With Follicular Lymphoma: First Steps

Most people expect a cancer diagnosis to be followed by treatment. In follicular lymphoma it often is not, and that reversal is the hardest part of the first week.

NCI calls watchful waiting a standard of care during the initial encounter. It means planned monitoring, with treatment held back until symptoms or disease progression appear.

The reason is arithmetic, not neglect. Follicular lymphoma is slow. NCI gives a median survival of 8 to 15 years even in advanced stages. It is also honest that advanced-stage disease is not cured by current treatments, and that relapse happens at a fairly steady rate over time, even after a complete response. Starting drugs sooner does not change that.

Three randomised trials compared watchful waiting with immediate chemotherapy. None found a difference in cause-specific survival or overall survival.

Two details from those trials are worth carrying with you. The median time before treatment was needed was 2 to 3 years. And one-third of the people who waited never needed treatment at all. Half of that third died of something else, and the other half were still free of progression after 10 years.

A separate group of 107 people with advanced-stage disease started with watchful waiting. Their treatment was delayed by a median of 55 months. Their results matched a similar group treated straight away with rituximab.

Waiting is not doing nothing. Ask exactly how often you will be examined and have blood tests, and write down the answer.

You may be given a FLIPI score. It uses five risk factors to sort people into outcome groups.

NCI reports that people with zero or one risk factor have a 10-year survival rate of 67 percent, and those with four or five have 36 percent. A revised version, FLIPI-2, swaps in beta-2-microglobulin and a lymph node larger than 6 cm.

Here is the part that gets misread. NCI states directly that these scores cannot be used to establish the need for therapy, or to predict how well treatment will work. Someone with a worrying FLIPI score may still be right to wait.

Options include rituximab on its own, rituximab with chemotherapy, lenalidomide with rituximab, obinutuzumab with or without chemotherapy, and maintenance rituximab afterwards.

The comparisons between them are less dramatic than they sound. In one trial of 534 people, R-CHOP, R-FM, and R-CVP gave an 8-year overall survival of 83 percent with no difference between them. Bendamustine with rituximab beat R-CHOP on median progression-free survival, 69 months against 31, with no difference in overall survival. It also caused less hair loss, fewer mouth ulcers, less nerve damage, and fewer infections.

Rituximab with lenalidomide, an approach that avoids cytotoxic drugs, matched rituximab plus chemotherapy at 6 years, with progression-free survival of 60 and 59 percent.

Two years of maintenance rituximab stretched median progression-free survival from 4.1 years to 10.5 years in the PRIMA study. Overall survival was no different. That trade is worth discussing rather than assuming.

Follicular lymphoma can change into a faster-growing lymphoma, usually diffuse large B-cell lymphoma. In a review of 325 people diagnosed between 1972 and 1999, the 10-year risk of this was 30 percent.

Median survival after transformation was 1 to 2 years, with 25 percent alive at 5 years. In that same series, advanced stage, a high-risk FLIPI, and watchful waiting were all linked to later transformation. That finding sits alongside the randomised trials showing no survival cost to waiting, and it is a fair thing to raise with your team.

The practical point is symptom-driven. Something growing fast is a reason to be seen, not a reason to wait for the next appointment.

Watchful waiting works because you are being watched. Between visits, you are the monitor, and these are the findings that should not wait for the next appointment.

• Call 911 or go to an emergency department if your face or neck swells with veins standing out on the chest, or breathing becomes hard at rest, or new leg weakness or trouble controlling your bladder or bowels appears. Enlarging nodes can press on structures that do not tolerate pressure.
• Call your care team straight away, day or night, if a temperature of 100.4°F (38°C) or higher appears while you are on rituximab or chemotherapy, since treatment lowers your defences against infection. CDC calls this a medical emergency, and it needs assessing at once. If you cannot reach them quickly, go to an emergency department and say you are on treatment.
• Call your care team within a day or two if a node visibly grows over a few weeks, or a new hard lump turns up. Something enlarging quickly is the main clue to transformation into a faster lymphoma, and it is a reason to be seen rather than reassured.
• Call your care team within a day or two if night sweats soak the bedclothes, fevers arrive with no infection behind them, or you lose more than a tenth of your body weight over roughly six months without trying.
• Call your care team within a day or two if pain settles in one spot and keeps worsening, or an arm or leg begins to swell.

Follicular Lymphoma, Active Surveillance vs Treatment, What Does Bone Marrow Involvement Mean?, and Lymphoma Treatment by Stage and Type.

• https://www.cancer.gov/types/lymphoma/hp/indolent-b-cell-lymphoma-treatment-pdq
• https://www.cdc.gov/cancer-preventing-infections/patients/fever.html