There are likely to be no B symptoms of disease, there may be some symptoms related to the swelling of involved lymph nodes usually located in the neck armpit or groin. Treatment of this stage of early disease is likely to be with a course of radiotherapy over 4 weeks. Chemotherapy is not normally given.

First line treatment for Advanced (stage II, III, IV)

These people are more likely to be unwell with symptoms of their lymphoma. There are two options for chemotherapy treatment:

• Combination chemotherapy – described in this section
• Bone marrow or stem cell transplant

The following are all standard drug regimes which may be used:

• R-CVP (rituximab and cyclophosphamide, vincristine and prednisolone)
• R-CHOP – (rituximab and cyclophosphamide, doxorubicin, vincristine and prednisolone)
• ESHAP – etoposide, cisplatin, ARA-C (cytarabine) and methylprednisolone
• Rituximab – a form of biological therapy
• IVE- ifosfamide etoposide epirubicin
• CVP (cyclophosphamide, vincristine and prednisolone)
• FAD (fludarabine, doxorubicin and dexamethasone)
• FMD (fludarabine, mitoxantrone and dexamethasone)
• Zevalin – a one off drug treatment used in conjunction with radiotherapy treatment

These chemotherapy regimes will usually be given over 2-8 three week cycles (6-28 weeks in total). Treatments effectively control disease in 60-80% of people although it almost always relapses. The median time to relapse is 3-4 years. Note each regime contains a steroid drug which may cause steroid related side effects.

Radiotherapy treatment may be given afterwards; this is likely to prolong treatment related fatigue.

People who are under 65 in poor health (who have poor performance status) may not be fit enough receive standard chemotherapy they are more likely to have the following treatment-:

• Chlorambucil +/- rituximab – Chlorambucil is a tablet form of chemotherapy which is often given by itself and is given intermittently over at least 6 months. It has few side effects.
• Rituximab alone

Relapsed disease (second line and subsequent line treatment)

When low grade lymphoma relapses further combination chemotherapy treatment is used. The chance of response and the duration of the response is usually less with each successive treatment. Eventually the disease is no longer responsive to chemotherapy and death occurs. Average survival from diagnosis with low grade NHL is 8-10 years.

Some people who obtain a 2nd or 3rd remission may go on to have high dose chemotherapy and bone marrow transplant if they are fit enough. If disease relapses again it may be kept under control for some time with further chemotherapy or radiotherapy. As a person has more treatments over time they are likely to collect disabling effects related to both the disease and its treatment.

Long term side effects of chemotherapy treatment

Of note because Non-Hodgkin Lymphoma affects young people the long term effects of treatment are particularly likely to affect this group and are summarised here:

• Infertility
• Secondary cancers
• Lung damage e.g. pulmonary fibrosis
• Heart disease
• Thyroid disorders

Those who do not respond to standard chemotherapy during the first course of chemotherapy treatment or whose lymphoma relapses after treatment are likely to go on to have high dose chemotherapy and Peripheral Blood Stem Cell Transplant (PBSCT) or bone marrow transplant treatment. The side effects of this treatment can be severe – refer to section on this treatment for guidance on disabling effects and duration of needs.

Radiotherapy

In early stage (stage 1 disease) - radiotherapy over 4 weeks is usually given. No needs are anticipated in relation to the disease. Occasionally side effects of radiotherapy may be very debilitating e.g. radiotherapy to the neck may cause severe problems with swallowing and pain necessitating naso-gastric tube feeding and possibly hospital admission. 10 year survival is around 80%. Around 50% of people treated will have a relapse of their lymphoma and go on to have further treatment.

In advanced low grade Non-Hodgkin Lymphoma small amounts of radiotherapy can be give to particularly troublesome nodes. Treatment is given daily over 1-2 weeks. Treatment is palliative and the aim is not to cause too many side effects.

Side effects

These are of note because Non-Hodgkin Lymphoma affects young people the long term effects of treatment are particularly likely to affect this group and are summarised here:

• Skin damage/pigmentation to treated area
• Lung damage
• Heart disease
• Secondary cancers
• Thyroid disorders

Steroid drugs

These include prednisolone, methylprednisolone and dexamethasone.

These drugs are used in short bursts during chemotherapy treatment. In general side effects are minimal. However if used for prolonged periods the following side effects are very common – significant weight gain, osteoporosis and diabetes mellitus. Rarer side effects include psychiatric problems ranging from depression and anxiety to major psychotic episodes.

• Side effects of chemotherapy
• Radiotherapy and Brachytherapy
• Problems in adults who had cancer treatment as children
• Bone marrow transplant
• Stages

Amended February 2009