Everything You Need to Know About Lymphoma

Lymphoma describes cancer that starts in the cells of your lymphatic (lymph) system. It occurs when certain white blood cells called lymphocytes become damaged. These mutant cells grow out of control and increase quickly. As they increase in number, the abnormal, damaged cells can gather to form tumors in your lymph nodes.

The two main types of lymphoma are non-Hodgkin lymphoma and Hodgkin lymphoma. They differ in the types of blood cells they affect. Treatment depends on your cancer type and how early you find it.

This article describes lymphoma types, symptoms, causes, stages, treatments, and outlook.

What Is the Lymphatic System?

The lymphatic system (also called the lymph system) is part of your immune system. Your lymphatic system includes the tissues and organs that produce, store, and carry white blood cells that fight infections and other diseases. Your lymphatic system includes the following:

• Lymph vessels: Thin tubes that carry lymph fluid away from body tissues and connect the organs and lymph nodes
• Lymph: A fluid similar in composition to blood plasma that contains lymphocyte cells
• Lymphocyte cells: White blood cells that fight infection and disease
• Lymph nodes: Bean-shaped organs located along the network of lymph vessels in your groin, underarm, neck, chest, and abdomen, that filter the lymph fluid as it circulates through your body
• Other lymphatic organs: Tonsils, spleen, thymus, bone marrow, and the digestive tract

Types of Lymphoma

There are two main types of lymphoma: non-Hodgkin lymphoma and Hodgkin lymphoma. However, they are easily confused because their names are so similar, and they both spread through the lymphatic system.

Non-Hodgkin Lymphoma

Non-Hodgkin lymphoma is the most common type of lymphoma. There are more than 60 distinct types of non-Hodgkin lymphoma. These diseases share similar characteristic that distinguish them from Hodgkin lymphoma.

The subtypes of non-Hodgkin lymphoma are categorized based on the characteristics of the cells they affect. You will be diagnosed and treated for the subtype you have.

The following criteria categorize non-Hodgkin lymphoma.

Type of cell involved:

• B-cell lymphoma: Develop from abnormal B-cell lymphocytes and are more likely to form the mutations that cause cancer
• T-cell lymphoma: Develop from abnormal T-cell lymphocytes

Rate of growth:

• Aggressive lymphomas: Develop symptoms sooner and require treatment faster
• Indolent: Lymphomas: advance slower and don't cause apparent symptoms in their early stages
  

Hodgkin Lymphoma

Hodgkin lymphoma is the least common type of lymphoma. It differs from non-Hodgkin lymphoma at the microscopic level. The lymph nodes in Hodgkin lymphoma contains giant cells called Reed-Sternberg cells. These cells have multiple nuclei and can be easily identified under a microscope. Reed-Sternberg cells do not occur in non-Hodgkin lymphoma.

There are four subtypes of Hodgkin lymphoma:

• Nodular sclerosis: Usually found in the chest and/or neck of children and young adults
• Mixed cellularity: Usually found in the abdomen of older adults
• Lymphocyte-rich: Usually found in men in their 30s
• Lymphocyte-depleted: Usually diagnosed in older adults and people with human immunodeficiency virus (HIV)

Lymphoma in Children

Lymphoma in children is rare. When the disease affects children, it can be either non-Hodgkin or Hodgkin lymphoma.

Non-Hodgkin lymphoma behaves differently in children than adults. This disease tends to be fast-growing and aggressive. It usually requires intensive therapy. Children under age 14 are more likely to have non-Hodgkin lymphoma.

Hodgkin lymphoma commonly affects children 15 and older. Children who have Hodgkin lymphoma usually develop one of the following subtypes:

• Nodular sclerosis: Affects 70% of children who have Hodgkin's lymphoma
• Mixed cellularity: Most commonly diagnosed in children younger than 10
• Nodular lymphocyte predominant: Most common in younger children

Lymphoma Symptoms

Many lymphoma symptoms are not specific to this disease and can easily be confused with other conditions. These symptoms can be subtle, allowing them to remain unrecognized for months or years before you realize you are ill. Some people don't have symptoms before diagnosis, often during a routine medical examination.

Lymphoma symptoms can vary based on the type of lymphoma that occurs. They can include the following:

• Lymphadenopathy (often painless swelling of the lymph nodes in your armpits, neck, or groin)
  
• Drenching night sweats
• Fever
  
• Pruritus (itching, especially after drinking alcohol or bathing)
• Anorexia (loss of appetite)
• Dyspnea (shortness of breath)
  
• Persistent fatigue
• Unintended weight loss
• Occasional lymph node pain after drinking alcohol
  

What Causes Lymphoma?

Lymphoma occurs when special white cells called lymphocytes become damaged, and the genes that control cell growth don't work properly. These damaged cells can grow uncontrollably or survive longer than normal. As the number of lymphocytes increases, they gather and collect to form tumors in the lymph nodes.

Non-Hodgkin Lymphoma Risk Factors

The following characteristics are known risk factors for non-Hodgkin's lymphoma:

• Age: Being age 60 or older
• Gender: Being male or female increases your risk, depending on the type of non-Hodgkin lymphoma
• Race: Being a Black American or Asian American
• Family history: Having a parent, child, or sibling with non-Hodgkin's lymphoma
• Chemical exposure: Exposure to the chemical benzene and certain weed- and insect-killing chemicals
• Radiation exposure: Being treated with radiation therapy for other cancers
• Weakened immune system: Having a weakened immune system due to a history of taking immunosuppressant drugs, HIV, or certain genetic syndromes like Wiskott-Aldrich syndrome (an immunodeficiency disorder)
• Infections that directly transform lymphocytes: Infections with human T-cell lymphotropic virus (HTLV-1), Epstein-Barr virus, human herpesvirus 8 (HHV-8)
• Infections that cause chronic immune stimulation: Infection with Helicobacter pylori (H. pylori), Campylobacter jejuni, and certain long-term infections with the hepatitis C virus (HCV)
• Body weight: Being overweight or experiencing obesity
  

Hodgkin Lymphoma Risk Factors

The following characteristics are known risk factors for Hodgkin's lymphoma:

• Epstein-Barr virus infection/mononucleosis: Epstein-Barr virus causes infectious mononucleosis, which raises your risk
• Age: Being in your 20s or over age 55
• Gender: Being male
• Family history: Having a brother or sister with Hodgkin's lymphoma, especially an identical twin
• Weakened immune system: Having an autoimmune disease or taking immunosuppressant drugs after an organ transplant

How Is Lymphoma Diagnosed?

Depending on your symptoms, your healthcare provider will use one or more of the following diagnostic tests to determine whether you have lymphoma. These tests can also help define the type and subtype of lymphoma.

Lymphoma is diagnosed using the following tests:

Physical examination: A physical examination for lymphoma involves palpating the lymph nodes (examining them by touch), a medical history, and discussing other symptoms.

Blood tests: The following types of blood tests can measure the number of red blood cells, white blood cells, and platelets, They can also identify specific enzymes or markers that indicate a diagnosis of lymphoma:

• Complete blood count (CBC)
• Beta-2 microglobulin (B2M),
• Lactate dehydrogenase (LDH)
• Erythrocyte sedimentation rate (ESR)
• Liver functions tests (LFTs) 
• HIV test
• Hepatitis B and hepatitis C tests

Imaging: If lymphoma is suspected, your healthcare provider may order one of the following imaging tests to look for swollen lymph nodes and learn how far your cancer spread:

• X-ray: Uses electromagnetic radiation to examine internal tissues, bones, and organs
• Computed tomography (CT) scan: Uses multiple X-rays to create a 3D image
• Magnetic resonance imaging (MRI): Uses a magnetic field and radio waves to produce two-dimensional or three-dimensional images of the structures inside your body
• Positron-emission tomography (PET) scan: Uses the injection of a short-acting radioactive substance called a radiotracer, which highlights cancer cells in pictures

Biopsies: A biopsy involves removing a tissue sample for examination in the lab. The following biopsies may be used to diagnose lymphoma:

• Excisional/incisional biopsy: Involves cutting through the skin to remove an entire lymph node (excisional biopsy) or a large portion of tissue (incisional biopsy)
• Core needle biopsy: Uses a large needle to extract tissue from a lymph node deep in your chest or abdomen
  

Stages of Lymphoma

Stages of lymphoma describe the extent to which cancer is spread and whether symptoms exist. This information helps your healthcare team determine your cancer's seriousness and the most appropriate treatment plan for your condition.

The main stages of non-Hodgkin and Hodgkin lymphoma are the same. They include the following:

Stage 1 Lymphoma:

• Cancer exists on one lymph node, a lymphoid organ.
• Or, cancer exists in one area of a single organ not part of your lymphatic system.

Stage 2 Lymphoma:

• Cancer exists on two lymph nodes, both on the same side of your diaphragm, the thin muscle between your lungs that separates your chest and abdomen).
• Or, cancer extends from one lymph node to a nearby organ.

Stage 3 Lymphoma:

• Cancer exists in lymph nodes on both sides (above and below) of your diaphragm.
• Or, cancer is in your lymph nodes above your diaphragm and your spleen.

Stage 4 Lymphoma:

• Cancer has spread outside your lymphatic system to at least one organ outside your lymphatic system, such as your bone marrow, liver, or lungs

Lymphoma can also be categorized further by adding letters to the stage number to provide further details (like stage 2A). These letters identify whether certain symptoms exist and how far your cancer has spread beyond your lymph nodes. These categories include the following:

• A: No symptoms
• B: Unexplained weight loss, night sweats, or fever
• X: Chest tumor that's considered bulky (larger than 10 centimeters in size)
• E: Extranodal cancer that spreads to other tissues or organs
• S: Cancer that's spread to your spleen

Lymphoma Treatment

With so many types, subtypes, and disease stages, lymphoma has a wide range of treatment options. The treatment you receive depends on the type of lymphoma you have, your disease stage, and your treatment goals.

Hodgkin Lymphoma Treatments

Several types of approaches and treatment combinations can be used for adults and children with Hodgkin lymphoma. These treatments include the following:

• Chemotherapy: Chemotherapy is the primary treatment for most cases of Hodgkin lymphoma. It involves the use of oral or injectable drugs to kill cancer cells. Chemotherapy may be used alone, with a group of drugs, or in combination with other treatments.
• Radiation therapy: Radiation therapy uses powerful, high-energy X-rays to slow or kill cancer cells outside your body. It is often used after chemotherapy as a two-step treatment.
• Immunotherapy: Immunotherapy involves the use of medicines to help your immune system better identify and destroy cancer cells. Two types of immunotherapy are used to treat Hodgkin lymphoma include monoclonal antibodies (human-made versions of your antibodies) and Adcetris (brentuximab vedotin), a human-made antibody that is modified to target and kill cancerous cells that express the CD30 protein, which is often found on the cells of classic Hodgkin lymphoma.
• High-dose chemotherapy and stem cell transplant: This treatment uses blood-forming cells from your own blood or bone marrow or that of a donor to offset the damage caused by high-dose chemotherapy.
  

Treating Non-Hodgkin Lymphoma

Treating non-Hodgkin lymphoma involves some of the same treatments used for Hodgkin's lymphoma and other options. These therapies include the following:

• Watchful waiting: Depending on your condition, your healthcare provider may advise postponing treatment for a period of watchful waiting to track your disease if you have a slow-growing form of lymphoma.
  
• Chemotherapy: Chemotherapy is the primary treatment for most cases of non-Hodgkin lymphoma.
• Immunotherapy: Immunotherapy can help your immune system fight lymphoma. Many treatments boost your immune system or use human-made versions of your immune system to kill lymphoma cells or slow their growth.
• Radiation therapy: Radiation therapy is often combined with chemotherapy. It can also be combined with immunotherapy in a treatment called radioimmunotherapy.
• Targeted drug therapy: Targeted drug therapy uses drugs that target the changes in lymphoma cells. These drugs interfere with the pathway that causes cancer to grow. They vary based on the type of proteins targeted.
• High-dose chemotherapy and stem cell transplant: A stem cell transplant restores bone marrow after treatment with high-dose chemotherapy.
• Surgery: Surgery is usually reserved for retrieving a tissue sample. Rarely, surgery may be used to treat lymphoma that has spread beyond.
  

Outlook for Lymphoma

Your outlook or prognosis is a forecast or prediction of how your healthcare provider expects your lymphoma to develop.

Your prognosis is calculated on the experiences of large groups of people over many years, not individual cases. Your experience and results with lymphoma can differ from the prognosis you receive.

The overall five-year relative survival rate for lymphoma varies based on the following categorizations of your disease:

• Hodgkin lymphoma: 89%
• Non-Hodgkin lymphoma: 74%

The following factors can affect these survival rates:

• Stage of lymphoma
• Type of cancer and where it is in your body
• Overall health
• Age
  

Living With Lymphoma: Support and Resources

Living with lymphoma requires more than the right medical treatment. A strong support system can make a difference in your physical and mental health and your treatment outcomes.

In addition to your family and friends, there are many resources for getting the practical lifestyle and emotional support you need to achieve the best possible outcomes. Caregivers can make all the difference in helping you maintain life as normal as possible while you undergo treatment and heal.

You may benefit from a palliative care support visit if you feel overwhelmed. This is a meeting with a team of specialists that includes a social worker, a nurse, and a healthcare provider who can address the full range of concerns involved in your cancer treatment.

Some of the most valuable resources involve others who understand what you are experiencing. Online or local groups can be valuable for emotional support, education, and resources. Check out lymphoma social media blogs by people sharing their cancer journeys to get different perspectives on dealing with your new reality.

Start by checking out the following organizations for resources, education, and social media connections:

• Leukemia and Lymphoma Society
• Lymphoma Research Foundation
• Cutaneous Lymphoma Foundation
• Cancer Care News (CCN)
• Cancer Survivors Network
  

Summary

Lymphoma is a group of blood cancers that affect your lymphatic system. These cancers form when white blood cells called lymphocytes grow and multiply abnormally. The damaged cells result in too many cells forming or living longer than they should. As their numbers grow, the impaired cells group to form tumors in your lymph nodes.

Lymphomas are categorized into two main groups: non-Hodgkin lymphoma and Hodgkin lymphoma. Non-Hodgkin lymphoma includes all lymphomas that don't have the large Reed-Sternberg cells found in Hodgkin lymphoma tissue.

Hodgkin lymphoma can often be cured. The outcomes for non-Hodgkin lymphoma vary based on which of its many forms occur.

Painless swelling in a lymph node is a common sign of this type of cancer. Other symptoms are often missed or thought to be signs of other ailments. Getting an early and correct diagnosis of disease symptoms can help improve your treatment outcomes.