Castleman disease

Title

Other Names:

Angiofollicular ganglionic hyperplasia; Angiofollicular lymph hyperplasia; Giant lymph node hyperplasia; Angiofollicular ganglionic hyperplasia; Angiofollicular lymph hyperplasia; Giant lymph node hyperplasia; Benign giant lymphoma See More

Categories:

Blood Diseases

Subtypes:

Multicentric Castleman Disease; Unicentric Castleman disease

Summary Summary

Castleman disease (CD) is a rare condition that affects the lymph nodes and related tissues. There are two main forms: unicentric CD and multicentric CD. Unicentric CD is a "localized" condition that is generally confined to a single set of lymph nodes, while multicentric CD is a "systemic" disease that affects multiple sets of lymph nodes and other tissues throughout the body.^[1]^[2] The exact underlying cause of CD is currently unknown; however, it is thought to occur sporadically in people with no family history of the condition.^[3] Treatment varies based on the form of the condition, the severity of symptoms and whether or not the affected person also has an HIV and/or human herpes virus type 8 (HHV-8) infection.^[4]

For more specific information about each form of CD, please visit GARD's unicentric Castleman disease and multicentric Castleman disease pages.

Last updated: 6/25/2015

Symptoms Symptoms

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms	Other Names	Learn More: HPO ID
30%-79% of people have these symptoms
Abdominal pain	Pain in stomach Stomach pain [ more ]	0002027
Anemia	Low number of red blood cells or hemoglobin	0001903
Elevated C-reactive protein level		0011227
Elevated erythrocyte sedimentation rate	High ESR	0003565
Fatigue	Tired Tiredness [ more ]	0012378
Follicular hyperplasia		0002729
Increased serum interleukin-6		0030783
Mediastinal lymphadenopathy	Swollen lymph nodes in center of chest	0100721
Weight loss		0001824
5%-29% of people have these symptoms
Abdominal distention	Abdominal bloating Abdominal swelling Belly bloating Bloating [ more ]	0003270
Abdominal mass		0031500
Cough	Coughing	0012735
Decreased mean corpuscular volume		0025066
Flank pain		0030157
Generalized lymphadenopathy	Generalized swelling of lymph nodes Swollen lymph nodes affecting all regions of the body [ more ]	0008940
Jaundice	Yellow skin Yellowing of the skin [ more ]	0000952
Nausea and vomiting		0002017
1%-4% of people have these symptoms
Anasarca		0012050
Dyspnea	Trouble breathing	0002094
Hematuria	Blood in urine	0000790
Intestinal obstruction	Bowel obstruction Intestinal blockage [ more ]	0005214
Myelofibrosis		0011974
Renal insufficiency	Renal failure Renal failure in adulthood [ more ]	0000083
Restrictive cardiomyopathy		0001723
Thrombocytopenia	Low platelet count	0001873
Ureteral obstruction		0006000

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Do you have more information about symptoms of this disease? We want to hear from you.

Last updated: 12/1/2019

Do you have updated information on this disease? We want to hear from you.

Cause Cause

The exact underlying cause of Castleman disease (CD) 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 CD. 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 CD.^[4]^[2]

It has also been found that a virus called human herpes virus type 8 (also known as HHV-8, Kaposi's sarcoma-associated herpesvirus, or KSHV) is present in many people with multicentric CD, specifically. HHV-8 is found in nearly all people who are HIV-positive and develop multicentric CD, and in up to 60% of affected people without HIV. The HHV-8 virus may possibly cause multicentric CD by making its own IL-6.^[4]^[2]

Last updated: 6/26/2015

Inheritance Inheritance

Although the exact underlying cause of Castleman disease is unknown, it is thought to occur sporadically in people with no family history of the condition.^[3]

Last updated: 6/26/2015

Research Research

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 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.

Organizations 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

Castleman Disease Collaborative Network
P.O. Box 3614
Paso Robles, CA 93447
Telephone: 610-304-0696
Fax: 877-991-9674
E-mail: info@castlemannetwork.org
Website: http://www.cdcn.org

Organizations Providing General Support

American Autoimmune Related Diseases Association (AARDA)
22100 Gratiot Avenue
Eastpointe, MI 48021
Toll-free: 800-598-4668
Telephone: 586-776-3900
Fax: 586-776-3903
E-mail: aarda@aarda.org
Website: https://www.aarda.org/

Do you know of an organization? We want to hear from you.

Learn More 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 Castleman disease. Click on the link to access this information.
The Mayo Clinic Web site provides further information on Castleman disease.
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 Castleman disease. Click on the link to view a sample search on this topic.

News & Events News & Events

News

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GARD Answers GARD Answers

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

Our daughter-in-law has Castleman's and our granddaughter is now hospitalized with symptoms that are consistent with what we have read about Castleman's. I am asking if it is a hereditary disease. See answer

Have a question? Contact a GARD Information Specialist.

References References

Jennifer R Brown, MD, PhD; Jon C Aster, MD; Nikhil C Munshi, MD. Unicentric Castleman's disease. UpToDate. November 2013; Accessed 6/24/2015.
Jon C Aster, MD; Jennifer R Brown, MD, PhD; Nikhil C Munshi, MD. Multicentric Castleman's disease. UpToDate. January 2015; Accessed 6/25/2015.
Castleman disease. Orphanet. January 2014; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=160.
Neetu Radhakrishnan, MD. Castleman Disease. Medscape Reference. January 2015; http://emedicine.medscape.com/article/2219018-overview.