Castleman Disease

What is Castleman disease?

Castleman disease, also known as giant lymph node hyperplasia and angiofollicular lymph node hyperplasia, is a rare disease that affects the body’s lymphatic system. The lymphatic system is the main part of the immune system and is composed of lymph vessels, lymph nodes, and lymphoid tissues. One of its roles is to absorb and transport large molecules, such as proteins and cellular debris. The lymph fluid containing these large molecules travels inside the lymph vessels to the lymph nodes. The lymph nodes are located throughout the body and are composed of a type of infection-fighting white blood cell called lymphocytes. The lymph nodes function to filter the lymph fluid and fight off bacteria/viruses. 
Castleman disease is considered a lymphoproliferative disorder because there is an overgrowth of cells in the lymphatic system. The disease can be localized to one set of lymph nodes (unicentric) or it can be spread throughout the body in multiple sets of lymph nodes and tissues (multicentric). Castleman disease is not a type of cancer, but the overgrowth of cells in multicentric Castleman disease is similar to that in cancer of the lymph nodes (lymphoma). People with Castleman disease are at increased risk for developing a lymphoma as well as some other cell-proliferation diseases. 
Unicentric Castelman disease is more common than multicentric. It typically affects the lymph nodes in the chest or abdomen and causes them to grow. When the disease affects the lymph nodes in places such as the groin, neck, or under the arms, people may feel a lump under their skin. 
Multicentric Castleman disease is a more serious form that affects multiple sets of lymph nodes and can even affect other organs that contain lymphoid tissue. It can also end up severely weakening one’s immune system. This makes these people more susceptible to life threatening infections. Multicentric Castleman disease sometimes develops in people infected with the human immunodeficiency virus (HIV). It is particularly serious in people who also have HIV and may worsen one’s prognosis. 
Risk factors
  • Having HIV/AIDS
  • Age: The average age of diagnosis is 35. Most who have the multicentric form are in their 50s and 60s. 
Castleman disease may be hard to diagnose at first because people often have no signs or symptoms. If your doctor suspects you may have Castleman disease they will likely complete a medical history and physical examination, paying close attention to the size and consistency of your lymph nodes. 
Some tests that may be done to diagnose Castleman disease include:
  • Imaging tests may be ordered to see if the lymph nodes, liver, or spleen are enlarged. A CT or MRI scan may be done of the neck, chest, abdomen, and pelvis. A positron emission tomography (PET) scan can be done to diagnose Castleman disease and determine if treatment is working.  
  • Blood and urine tests can help rule out other infections and diseases. They can also tell if a person has anemia or abnormalities in blood proteins which can be signs of Castleman diease. 
  • Lymph node biopsy may be done to differentiate Castleman disease from another lymphatic tissue disease. This test is done by taking a sample of tissue from an enlarged lymph node in order to be examined.
Treatment can depend on whether you have unicentric or multicentric Castleman disease. 
Some potential treatments for unicentric Castleman disease includes:
  • Surgery to remove the enlarged lymph node
  • Medication to shrink the lymph node
  • Radiation therapy 
Treatment options for multicentric Castleman disease include:
  • Monoclonal antibodies
  • Chemotherapy
  • Coritcosteroids
  • Antiviral drugs
  • Surgery to remove an enlarged spleen 
  • Immunotherapy
  • Unicentric Castleman disease is more common than the multicentric form
  • There are approximately 6,500-7,700 new cases of Castleman disease each year in the U.S.
  • Unicentric Castleman disease is more common in 20-30 year olds while the multicentric form is more common in 40-60 year olds
  • Multicentric Castleman disease accounts for 1 in 4 cases of Castleman disease
about castleman disease

  • "Castleman disease." Mayo Clinic. Mayo Foundation for Medical Education and Research, 27 Apr. 2014. Web. 25 Aug. 2017.
  • “What Is Castleman Disease?” American Cancer Society, 23 May. 2016. Web. 25 Aug. 2017. 
  • “About Castleman Disease.” Castleman Disease Collaborative Network. CDCN, n.d. Web. 25 Aug. 2017. 
  • “Castleman Disease.” National Organization for Rare Disorders. NORD, n.d. Web. 25 Aug. 2017.
  • “Castleman disease.” National Institutes of Health National Center for Advancing Translational Sciences. Genetic and Rare Diseases Information Center, n.d. Web. 25 Aug. 2017. 
  • “What Is The Lymphatic System?” National Lymphedema Network, n.d. Web. 25 Aug. 2017.  


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