Diagnosis and Prognosis

Diagnosis

The majority of follicular lymphoma patients have no noticeable symptoms when they are diagnosed, apart from one or more swollen lymph nodes. As a general rule, firm or hard nodes are more worrying than soft nodes; nodes that grow over time or remain in place more than a few weeks are more worrying than nodes which quickly shrink and disappear; and nodes that are more than 2 cm long are more worrying than nodes that are less than 2 cm long (by the same token, nodes less than 1 cm long are even less worrying, and are usually simply dismissed as normal by doctors).

Swollen lymph nodes are extremely common and almost always meaningless diagnostically. For this reason, doctors often prefer to observe them for several weeks or even months, and may prescribe antibiotics to take care of any underlying infection. If the nodes remain enlarged, the first test is typically a complete blood count (CBC). The CBC may rule out late-stage disease as well as a number of related conditions (such as leukemia), but it cannot definitively rule out follicular lymphoma because this disease does not necessarily affect circulating levels of blood cells. Indeed, many lymphoma patients anecdotally report having normal CBC reports throughout much of their diagnosis and treatment.

For this reason, a doctor who suspects malignancy will likely follow up with more advanced imaging scans. An ultrasound can be used to search for swollen lymph nodes in the soft tissue of the neck and the abdomen. A chest X-ray is commonly ordered to search for nodes in the chest area; this can turn up affected nodes around the lungs (potentially indicating other causes, like lung cancer, sarcoidosis, or tuberculosis), or enlargement of the mediastinum, a large mass of lymphatic tissue located in the chest.

Doctors also have access to more advanced tests. These include Computerized Tomography (CT scans or "CAT" scans), which are a long series of X-ray images assembled by computer into a three-dimensional image of the body; PET scans; and MRI scans. Because advanced services are rationed in most provinces and waiting lists are long, CT scans may be the easiest and quickest to get. What scans are publicly funded, and whether there is a (lengthy) wait list, depends upon the province.

The most important diagnostic procedure, however, is a biopsy of an affected node, which can detect the presence of malignant cells (or, in healthy individuals, the lack of such cells). Usually a minimally invasive procedure known as a fine needle aspiration (FNA) biopsy is performed first. If the results of the FNA are uncertain or abnormal, this may be followed by an open biopsy, removing the entire node for analysis. The surgeon or specialist will usually target the largest easily accessible node, on the principle that if malignancy is present at all, it should be most easily detectable in the largest lymph nodes.
  • When to Worry About a Swollen Lymph Node
  • Lymph Nodes and Other Body Sites Lymphoma Can Affect
  • About the Complete Blood Count (CBC)
  • How Ultrasound Works
  • How CT Scans Work
  • Canadian Waiting Lists for Scans
  • Biopsy for Lymphoma


Staging

Once cancer is diagnosed, it is staged. This is a process by which doctors estimate how far the disease has already progressed. Traditionally, follicular lymphoma is staged according to the Ann Arbor system, which has four parts:
  • Stage I: localized disease. One node or group of nodes is involved.
  • Stage II: lymphoma has spread to two or more regions, on one side of the diaphragm (e.g. upper or lower body)
  • Stage III: lymphoma has spread to lymph node groups throughout the body
  • Stage IV: lymphoma has spread to extranodal sites, including the bone marrow.
In addition to the above, follicular lymphoma may be delineated "A," meaning no symptoms (apart from swollen lymph nodes), or "B," meaning constitutional symptoms are present (such as a fever, drenching night sweats, or unexplained weight loss). Finally, follicular lymphoma is also assigned a grade of 1-3 based upon cellular characteristics related to the degree of aggressiveness of the cancer. Hence a particular disease may be referred to as "Stage III," for instance, although it could also be fully (and properly) referred to as "grade 2, Stage IIIa." Certain grade 3 follicular lymphomas are treated as aggressive lymphomas, and these are very uncommon.

Staging usually makes use of advanced scanning technology to search for lymph nodes around the body, such as a CT scan. In many provinces PET scans are also used. However, PET scan services vary around the country. In Ontario, for instance, PET scans for lymphoma are only publicly funded after chemotherapy has already begun.


Prognosis

A prognosis is an educated guess about how a disease will progress in the future. Traditionally, the prognosis for follicular lymphoma is poor. The disease grows slowly, but conventional cytotoxic chemotherapy has little effect on overall survival rates, with median survival (50% survival) of about 10 years. Some patients (about 3% per year) experience transformation to diffuse large B-cell lymphoma, which is aggressive and has a poorer prognosis.

It is important to know, however, that there are no estimates for how long a newly diagnosed patient will survive. There can't be. By definition, a 10-year survival rate means how many people diagnosed 10 years ago are still alive. In the meantime, rituximab has become standard therapy and a range of other new therapies have also become available. There is every reason to believe that the life expectancy of follicular lymphoma sufferers has grown substantially in a fairly brief period of time.

There are several methods used to make prognostic guesses about follicular lymphoma, including the International Prognostic Index (IPI), the Follicular Lymphoma International Prognostic Index (FLIPI), and the Second Follicular Lymphoma International Prognostic Index (FLIPI-2). The FLIPI system identifies five significant factors:
  1. Advanced disease (Stage III or Stage IV)
  2. Elevated serum LDH in blood
  3. Age greater than 60 years old
  4. Five or more affected lymph node regions
  5. Low hemoglobin
Each factor is worth one point. People with 0-1 points are low risk (5-year survival of about 90%), 2 points are medium risk (about 70%), and 3-5 points are high-risk (about 50%). Note that these survival figures, and the FLIPI study itself, were developed using pre-rituximab patients.

Once lymphoma has been diagnosed, an oncologist will discuss options for treatment. Lymphoma treatment is covered by provincial cancer care programs in all Canadian provinces, although the precise treatments funded by these programs vary, as do waiting lists and other service issues.