National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Unicentric Castleman disease


Other Names:
Unicentric angiofollicular ganglionic hyperplasia; Unicentric angiofollicular lymph hyperplasia; Localized Castleman disease
Categories:
This disease is grouped under:
Unicentric Castleman disease (UCD) is a rare condition that affects the lymph nodes and related tissues. It is a form of Castleman disease that is "localized" to a single set of lymph nodes (as opposed to multicentric Castleman disease which has more widespread effects). UCD often starts as an enlarged lymph node. Depending on the location of this enlarged node, some people will have no additional features of the condition, while others will develop symptoms when it pushes on nearby organs and/or tissues. The exact underlying cause of UCD is currently unknown. Treatment typically includes surgical removal of the affected lymph node.[1][2][3]
Last updated: 6/24/2015
Some people with unicentric Castleman disease (UCD) have no signs or symptoms of the condition. When present, symptoms are often nonspecific and blamed on other, more common conditions. UCD is "localized" (only affects one area of the body) and often starts as an enlarged lymph node. Depending on the location of this enlarged node, some people may experience the following symptoms if it pushes on nearby organs and/or tissues:[4][1][3]
  • A feeling of fullness or pressure in the chest or abdomen
  • Trouble eating or breathing
  • Unintended weightloss
  • Less commonly, fever, night sweats and weakness

 

Last updated: 6/23/2015
The exact underlying cause of unicentric Castleman disease (UCD) is poorly understood. However, some scientists suspect that an increased production of interleukin-6 (IL-6) by the immune system may contribute to the development of UCD. IL-6 is a substance normally produced by cells within the lymph nodes that helps coordinate the immune response to infection. Increased production of IL-6 may result in an overgrowth of lymphatic cells, leading to many of the signs and symptoms of UCD.[5][2]

Last updated: 6/24/2015
Although the exact underlying cause of unicentric Castleman disease (UCD) is unknown, it is thought to occur sporadically in people with no family history of the condition.[6]
Last updated: 6/24/2015
Some people affected by unicentric Castleman disease (UCD) do not have any specific signs or symptoms. In these cases, the enlarged lymph node may be discovered incidentally (by chance) during a routine physical examination or when an imaging study is ordered to investigate a different medical condition. If UCD is suspected, the following tests may be recommended to help establish the diagnosis and rule out other conditions that cause similar features:[3][2]
  • Blood tests can be ordered to evaluate the levels of Interleukin-6 (IL-6) and other substances in the body, which can be elevated in people with UCD. They can also be helpful in ruling out other autoimmune conditions and infections that are associated with similar signs and symptoms
  • Imaging studies (such as a CT scan, PET scan, MRI scan, and/or ultrasound) can help identify enlarged lymph node(s) and other health problems
  • Lymph node biopsy is usually recommended to confirm the diagnosis
Last updated: 6/24/2015
Unicentric Castleman disease (UCD) can usually be cured by surgically removing the enlarged lymph node. If surgery isn't an option or the lymph node can not be removed completely, radiation therapy may be recommended to shrink and/or destroy the affected tissue.[1][2][3]

Last updated: 6/24/2015
The long-term outlook (prognosis) for people with unicentric Castleman disease (UCD) is generally excellent. In many cases, the condition can be cured by surgically removing the affected lymph node. In fact, one study found that disease-free survival in people with UCD following surgery was approximately 93%.[3]
Last updated: 6/24/2015

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Unicentric Castleman disease. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.
  • The Research Portfolio Online Reporting Tool (RePORT) provides access to reports, data, and analyses of research activities at the National Institutes of Health (NIH), including information on NIH expenditures and the results of NIH-supported research. Although these projects may not conduct studies on humans, you may want to contact the investigators to learn more. To search for studies, enter the disease name in the "Text Search" box. Then click "Submit Query".

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

Organizations Providing General Support

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • The American Cancer Society provides detailed information about Unicentric Castleman disease. Click on the link to access this information. 
  • The Castleman Disease Collaborative Network, a global initiative dedicated to accelerating research and treatment for Castleman disease, provides information about Unicentric Castleman disease.
  • MayoClinic.com provides an information page for this topic. Click on the link to view this information.
  • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Unicentric Castleman disease. Click on the link to view a sample search on this topic.

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  1. Castleman Disease. American Cancer Society. July 2014; http://www.cancer.org/cancer/castlemandisease/detailedguide/index.
  2. Jennifer R Brown, MD, PhD; Jon C Aster, MD; Nikhil C Munshi, MD. Unicentric Castleman's disease. UpToDate. November 2013; Accessed 6/24/2015.
  3. Neetu Radhakrishnan, MD. Castleman Disease. Medscape Reference. January 2015; http://emedicine.medscape.com/article/2219018-overview.
  4. Castleman disease. Mayo Clinic. August 2014; http://www.mayoclinic.org/diseases-conditions/castleman-disease/basics/definition/con-20031703?p=1.
  5. Castleman's Disease. NORD. December 8, 2010; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/532/viewAbstract. Accessed 10/1/2013.
  6. Castleman disease. Orphanet. January 2014; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=160.