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Disease Profile

Unicentric Castleman disease

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

All ages

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ICD-10

D47.7

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Unicentric angiofollicular ganglionic hyperplasia; Unicentric angiofollicular lymph hyperplasia; Localized Castleman disease

Categories

Blood Diseases; Rare Cancers; Viral infections

Summary

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]

Symptoms

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

 

Cause

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]

Diagnosis

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

Treatment

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]

Organizations

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

      Learn more

      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.

          References

          1. Castleman Disease. American Cancer Society. July 2014; https://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; https://emedicine.medscape.com/article/2219018-overview.
          4. Castleman disease. Mayo Clinic. August 2014; https://www.mayoclinic.org/diseases-conditions/castleman-disease/basics/definition/con-20031703?p=1.
          5. Castleman's Disease. NORD. December 8, 2010; https://www.rarediseases.org/rare-disease-information/rare-diseases/byID/532/viewAbstract. Accessed 10/1/2013.
          6. Castleman disease. Orphanet. January 2014; https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=160.

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