Non-Hodgkin Lymphoma (Lymphoid Neoplasms)

To make a definite diagnosis of NHL, doctors need to collect a sample of the affected lymph node. This procedure is called a biopsy. Biopsies where an entire lymph node or tumor is removed (called excisional biopsies) are preferred to ensure there is enough sample to determine the type of lymphoma. The biopsy is typically studied by a pathologist (doctor who specializes in the diagnosis of diseases by studying the cells from a patient’s body fluids and tissue samples) and preferably a hematopathologist (pathologist who has undergone additional training in the diagnosis of blood cancers, including lymphoma) who is experienced in diagnosing lymphoma. There are multiple subtypes of NHL, some very uncommon, and additional tests may be needed to make an accurate diagnosis. Determining the exact NHL subtype helps to identify the appropriate treatment options for the patient.

Figure 3: Staging of NHL according to the Lugano system. NHL, non-Hodgkin lymphoma

Stage I:

Involvement of a single lymph node or group of adjacent nodes

Stage II:

Involvement of two or more groups of lymph nodes on the same side of the diaphragm (muscle that separates the chest from the abdomen)

Stage III:

Involvement of lymph nodes on both sides of the diaphragm, or Involvement of lymph nodes above the diaphragm plus spleen involvement

Stage IV:

Widespread disease in lymph nodes, bone marrow, and organ involvement, such as liver or lungs

After a diagnosis of NHL, it is important to determine if and how far the lymphoma has spread. This process is called staging, and it uses the results of the different tests (such as biopsies and scans) to determine the severity of the disease and the appropriate treatment. The Lugano staging system is used for most NHLs (except CLL/SLL) and is depicted in Figure 3 above. This system categorizes NHL from Stage I (least severe) to IV (most severe), based on whether the disease is restricted to a single group of lymph nodes, has spread to other lymph nodes, or has reached the bone marrow (the spongy tissue inside the bones) and/or other organs (like the liver or lungs).

To stage a lymphoma, the patient might need imaging tests such as abdominal and chest CT scans or a positron emission tomography (PET) scan. A CT scan allows the physician to see inside the chest and abdomen, locating the tumor. A PET scan is a form of imaging that uses a special dye to locate the lymphoma cells in the body. Other staging tests may include a bone marrow biopsy, spinal tap (lumbar puncture, a procedure where a small needle is inserted into the back and spinal fluid is withdrawn), endoscopy/colonoscopy (medical procedures where an instrument is introduced into the body to give a view of the stomach or colon, respectively), and magnetic resonance imaging (MRI, a type of scan that uses a strong magnet and radio waves to produce detailed images of the inside of the body). Physicians may also request blood tests and an echocardiogram (a type of scan that uses sound waves to produce images of the heart and nearby blood vessels) to help evaluate overall health and risks with chemotherapy

The characteristics that may make a person more at risk of developing any type of disease are called risk factors. Having one or more risk factors for NHL does not mean a person will develop NHL. People with a family history of NHL appear to be at slightly higher risk of developing the disease, often the same subtype. Nonetheless, the likelihood of two first-degree relatives having NHL remains very small. In fact, most people with the known risk factors never develop NHL. The causes of NHL in most cases remain unknown. Known risk factors for NHL include:

• A weakened immune system caused by an inherited immune disorder (for example, hypogammaglobulinemia, ataxia-telangiectasia or Wiskott-Aldrich syndrome) or infection with human immunodeficiency virus (HIV).
• An autoimmune disease (for example, Crohn’s disease, rheumatoid arthritis, systemic lupus erythematosus, or psoriasis).
• Treatment for autoimmune diseases (the body’s immune system attacks its own healthy cells), especially with methotrexate and drugs that target a protein called tumor necrosis factor.
• Treatment with certain drugs used after organ transplantation.
• Infections with certain viruses (for example, Epstein-Barr virus [EBV], human T-cell lymphotropic virus type 1 [HTLV-1], human herpes virus 8 [HHV-8], or hepatitis C virus [HCV]).
• Infection with the bacteria Helicobacter pylori, Campylobacter jejuni, or Chlamydia psittaci.
• Older age—NHL is much more common in adults older than 60 years, although it may develop in children and adults of all ages.
• Males have slightly higher incidence rates (number of new cases) of NHL than women.
• Exposure to certain chemicals (benzene), herbicides (Agent Orange) and pesticides, and some chemotherapy drugs used to treat other cancers.
• Treatment with radiation therapy for other cancers, including NHL.

The classification of NHL is complicated and has evolved over the years. NHL subtypes are grouped according to which kind of white blood cell is affected (B cells or T cells) and how quickly the cancer grows (aggressive or indolent). There are more subtypes of NHL than those listed here. Please consult with a physician if you are not sure of your subtype. Knowing as much as possible about your NHL subtype, treatment options and their potential side effects can empower you to take charge of your health and better communicate with your physician. The following table includes blood diseases in the current World Health Organization (WHO) classification. This table includes chronic lymphocytic leukemia (CLL) and small lymphocytic lymphoma (SLL), which are different forms of the same disease.

Table 1: Types of NHL by cell type and growth pattern.

The charts below shows how common or uncommon B- and T-cell NHLs are.

Figure 1: Relative frequencies of B-cell NHL in the United States. Percentages are based on the National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) data, 2008-2017. SEER Program provides information on cancer statistics. Some very rare types are not shown in the graph. NHL, non-Hodgkin lymphoma.

Figure 2: Relative frequencies of T-cell NHL in the United States. Percentages are based on the National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) data, 2008-2017. Some very rare types are not shown in the graph. NHL, non-Hodgkin lymphoma.

Keeping your information in one location can help you feel more organized and in control. This also makes it easier to find information pertaining to your care and saves valuable time. The Foundation’s Lymphoma Care Plan document organizes information on your health care team, treatment regimen, and follow-up care. You can also keep track of health screenings and any symptoms you experience to discuss with your health care provider during future appointments. The Lymphoma Care Plan document can be accessed by visiting lymphoma.org/publications.