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Paraneoplastic Syndromes and Lymphoma

Posted on December 22, 2021
Medically reviewed by
Todd Gersten, M.D.
Article written by
Brooke Dulka, Ph.D.

Paraneoplastic syndromes refer to a group of rare disorders that people with cancer can develop as a result of their disease. Specifically, paraneoplastic syndromes occur when the antibodies in the immune system begin to attack normal cells in the body in response to cancerous tumors (also called neoplasms). Paraneoplastic syndromes are also considered to be distant effects of cancerous tumors.

These paraneoplastic syndromes happen in the context of different types of cancer, including lymphoma. Understanding the connection between paraneoplastic syndromes and lymphoma is important because the presence of paraneoplastic syndromes can make the prognosis of the underlying lymphoma worse, particularly if the syndromes are left untreated.

What Are Paraneoplastic Syndromes?

Paraneoplastic syndromes are disorders caused by abnormal immune system responses to cancerous tumors (neoplasms). There are many different types of paraneoplastic syndromes, but their causes are relatively similar.

In general, paraneoplastic syndromes are caused by the body’s immune system attacking itself (called an autoimmune response). For this reason, paraneoplastic disorders are autoimmune diseases.

Paraneoplastic syndromes have the potential to affect many parts of the body, including the nervous system (brain, spinal cord, and nerves), the hormonal (or endocrine) system, skin, blood, and joints. People with cancer often experience fever, night sweats, loss of appetite, and weight loss as manifestations of paraneoplastic syndromes.

An example of one paraneoplastic syndrome associated with lymphoma is paraneoplastic cerebellar degeneration, a disease in which a brain region known as the cerebellum begins to deteriorate — meaning certain brain cells are being destroyed.

Many symptoms are associated with paraneoplastic syndromes. Commonly, these symptoms can include fever, night sweats, weight loss, and loss of appetite. Other general symptoms can include:

  • High blood pressure
  • Dizziness
  • Difficulty with speech
  • Weakness
  • Weight gain
  • Double vision
  • Loss of coordination or sensation
  • Slower reflexes
  • Flushing (redness) of the skin
  • Benign (noncancerous) skin growths
  • Itching
  • Neurological symptoms related to the central nervous system (brain and spinal cord)
  • Neuropathy (damage to nerves)

How Prevalent Are Paraneoplastic Syndromes in Lymphoma?

In general, paraneoplastic syndromes are thought to develop in as many as 20 percent of people who have any type of cancer. The syndromes occur most often in middle-aged people who have lung, ovarian, breast, or lymphatic cancer (lymphoma).

Although paraneoplastic syndromes can be fairly common in people with lymphoma, there isn’t much data on how prevalent these diseases are. Most of the research done has been in the context of paraneoplastic disorders of the nervous system.

In one small study of people with either Hodgkin lymphoma or non-Hodgkin lymphoma, the most commonly associated paraneoplastic neurological syndrome was cerebellar degeneration, present in 21 out of 53 cases. The prevalence of paraneoplastic cerebellar degeneration was higher in Hodgkin lymphoma (16 out of 24 cases) compared to non-Hodgkin lymphoma (5 out of 29 cases).

Paraneoplastic syndromes of the peripheral nervous system (such as demyelinating polyradiculopathy) were more common in people with non-Hodgkin lymphoma. Demyelinating polyradiculopathy is a disorder in which the body’s immune system attacks the myelin (fatty sheath) that insulates and protects the body’s nerves.

Other Paraneoplastic Syndromes Associated With Lymphoma

Other paraneoplastic syndromes have also been observed in people with lymphoma.

For example, autoimmune cytopenias are a common paraneoplastic syndrome in lymphomas. Autoimmune cytopenias are a related group of disorders that affect blood cells. One study observed that certain autoimmune cytopenias can all occur in people with Hodgkin lymphoma:

Many other paraneoplastic syndromes have also been observed in the context of lymphoma. However, most published information is in the form of case reports (a single instance of the syndrome) or in reports of a small number of people.

For instance, one case study of a person with non-Hodgkin lymphoma observed a paraneoplastic disease known as opsoclonus-myoclonus syndrome. This syndrome affects the nervous system and involves rapid, multidirectional eye movements (opsoclonus), quick, involuntary muscle jerks (myoclonus), uncoordinated movement (ataxia), irritability, and sleep disturbance.

Treatment and Outlook of Paraneoplastic Syndromes

People may notice the symptoms of a paraneoplastic syndrome before they notice any symptoms from underlying cancer. Prompt and effective diagnosis and treatment of paraneoplastic syndromes is important because these syndromes can affect outcomes related to lymphoma.

If someone has paraneoplastic syndromes, the syndromes can be treated in many different ways, such as treating underlying cancer. As there are also many types of paraneoplastic syndromes, the treatment of paraneoplastic symptoms varies depending on which type it is and what part of the body is being attacked.

Because these syndromes are related to inflammation and the autoimmune system, treatment options can include:

  • Corticosteroids — Reduce inflammation
  • Intravenous immunoglobulins — Boost healthy antibodies
  • Immunosuppressants — Manage the immune system’s response
  • Plasmapheresis — Reduces the amount of antibodies in the blood
  • Speech and physical therapy — Helps people speak and move better

As paraneoplastic syndromes vary from one person to the next, the prognosis (outlook) of these syndromes will also vary. In some cases, symptoms can be easily managed. In other cases, they can be life-threatening.

Because the presence of paraneoplastic syndromes can make the prognosis of underlying cancer worse, it is important to recognize paraneoplastic syndromes when they occur. Paraneoplastic antigens may even be able to serve as early biomarkers for some types of cancer. If you are experiencing new or unusual symptoms, talk to your doctor as soon as possible.

Talk With Others Who Understand

MyLymphomaTeam is the social network for people with lymphoma. More than 10,000 members come together to ask questions, give advice, and share their stories with others who understand life with lymphoma.

Are you or someone you care for living with lymphoma and a paraneoplastic syndrome? Share your experience in the comments below, or start a conversation by posting on MyLymphomaTeam.

All updates must be accompanied by text or a picture.
Todd Gersten, M.D. is a hematologist-oncologist at the Florida Cancer Specialists & Research Institute in Wellington, Florida. Review provided by VeriMed Healthcare Network. Learn more about him here.
Brooke Dulka, Ph.D. is a freelance science writer and editor. She received her doctoral training in biological psychology at the University of Tennessee. Learn more about her here.

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