Unicentric Castleman Disease (UCD)

Diseases of the immune system

Rare Disease Classifications:

ORPHA:93685 Unicentric Castleman disease

ICD-11: 4B2Y Other specified disorders involving the immune system

IMPORTANT INFORMATION

The information on the Rare Awareness Rare Education (RARE) Portal is intended for educational purposes only and does not replace professional advice.

Rare diseases typically display a high level of symptom complexity and variability. Individuals diagnosed with the same rare disease may be impacted differently and each person’s experience is unique. Please seek support from qualified healthcare professionals to learn more about the most suitable care and support options for you.

There are currently no known organisations in Australia specifically for this rare disease. If you know of any rare disease organisation/s supporting people living with Castleman disease, please let us know via the Contribute page. If you are interested in starting an Australian organisation for people living with Castleman disease, please see Engaged, Ethical and Effective: A Guide for Rare Disease Organisation Leaders in Australia.

If you are aware of any additional information that may benefit stakeholders with an interest in this page, or if you notice any broken links or inaccurate information, please let us know via the Contribute page.

Emergency Management

There may be special considerations for the emergency management of individuals living with Castleman disease presenting to emergency departments.

Clinical Care Guidelines

We are not aware of any clinical care guidelines for Castleman disease in Australia. If you know of any relevant care guidelines, please let us know via the Contribute page.

The following guidance is available from international experts outside Australia; however, there may be information that is not relevant or applicable to the Australian context, and may not be up to date:

The management of Castleman disease – a ‘Good Practice Paper’ containing recommendations where there is limited evidence but a degree of consensus or uniformity is likely to be beneficial to patient care; compiled according to the British Society for Haematology (BSH) process and published in 2021
International evidence-based consensus diagnostic and treatment guidelines for unicentric Castleman disease; published in 2020 by 42 experts from 10 countries that convened to establish consensus recommendations

Synonyms

CD, UCD, angiofollicular lymph node hyperplasia, angiomatous lymphoid, angiofollicular ganglionic hyperplasia, Castleman tumour, giant benign lymphoma, giant lymph node hyperplasia, hamartoma of the lymphatics

Summary

Unicentric Castleman disease (UCD) is a type of Castleman disease that is characterised by the enlargement of a lymph node or a cluster of lymph nodes, with distinct features that are only visible when examined under a microscope.^1-3. Lymph nodes are part of the immune system and are the home base of immune cells. Unlike multicentric Castleman disease, which involves multiple lymph nodes and can affect other organs, UCD is confined to one area. In most cases, the symptoms are caused by the enlarged lymph node affecting nearby tissues and organs and may resolve with the removal of the affected lymph node.^2,4,5

Useful Links for Professionals

Orphanet: Castleman Disease

Personal Stories

Castleman disease varies between individuals, and each person’s experience is unique.

If you would like to share your personal story with Rare Voices Australia and have it included on the RARE Portal, please visit Rare Voices Australia: Share Your Story.

Symptoms

Symptoms of unicentric Castleman disease (UCD) can vary between individuals. People with UCD usually experience no symptoms.^2,4-6 The affected lymph node/s generally appear as a non-painful lump. Occasionally mild fatigue, aches and pains, and mild anaemia can occur. UCD can cause more severe symptoms when the lymph nodes grow in a way that it physically impacts on nearby organs, blood flow or nerves. The symptoms vary depending on what the enlarged lymph nodes impact.

Please speak to your medical team to learn more about the symptoms and complications of unicentric Castleman disease.

Cause/Inheritance

The cause of unicentric Castleman disease (UCD) is unknown.^1,2,4,6 There are no known risk factors.

Diagnosis

Diagnosis of unicentric Castleman Disease (UCD) may be made based on the results of an excisional lymph node biopsy as well as further tests to rule out other conditions.⁴

An excisional lymph node biopsy involves the removal of the entire lymph node (either whilst awake under local anaesthetic or asleep using general aesthetic). The lymph node is then examined under a microscope to look for the characteristic changes that are associated with Castleman disease.

A computerised tomography (CT) or positron emission tomography–computed tomography (PET-CT) scan is often performed to look for the presence of other affected lymph nodes in order to differentiate between UCD (only one or one cluster of affected lymph node) and multicentric Castleman disease (multiple affected lymph nodes).^4,6

Differential diagnosis (to rule out other conditions) includes multicentric Castleman disease, Hodgkin lymphoma, non-Hodgkin lymphoma, thymomas, systemic lupus erythematous, autoimmune diseases, and acute viral infections including Epstein-Bar virus and human immunodeficiency virus infections.^2,4-7

Please speak to your medical team to learn more about the available diagnostic pathways for Castleman Disease.

Treatment

Treatment of unicentric Castleman disease (UCD) may involve removal of the affected lymph node/s. Complete removal of the affected lymph node/s can usually resolve the symptoms associated with UCD. In some cases, removal of the node is not possible and may be treated with medications or radiation therapy to shrink the lymph node.⁵ Medications may also be used to shrink the lymph node to a size or position that allows it to then be surgically removed.

Clinical Care

Healthcare professionals involved in the treatment of unicentric Castleman disease may include general practitioners (GP), surgeons, paediatricians, haematologists, and oncologists.^2,4,8 The need for different healthcare professionals may change over a person’s lifetime and extend beyond those listed here.

Research and Data

Castleman Disease Collaborative Network: Physicians & Researchers has information on relevant research and resources for Castleman disease.

Please visit Australian Clinical Trials to learn about clinical trials for Castleman disease in Australia; there may not be any clinical trials currently available.

Information regarding clinical trials for Castleman disease in other countries can be found at ClinicalTrials.gov; there may not be any clinical trials currently available.

It is best to discuss your interest in any clinical trials with your medical team to determine suitability and eligibility.

Rare Disease Organisation(s)

There are currently no known organisations for Castleman disease in Australia. If you are aware of any Castleman disease organisations in Australia, please let us know via the Contribute page.

Global organisations
Castleman Disease Collaborative Network
Website: https://cdcn.org/
Contact form: https://cdcn.org/contact/

The Castleman Disease Collaborative Network (CDCN) is an organisation focused on improving the understanding, diagnosis, and treatment of Castleman disease. They provide information about the disease, promote research, facilitate collaboration among scientists and clinicians, and provide support to patients and families affected by Castleman disease.

Please note that RVA does not necessarily monitor or endorse each group/organisation’s operational governance.

For information on available government and social services that provide support for individuals with a rare disease, please visit the National and State Services pages.

Mental Health

Please visit the ‘Mental Health’ sections listed on the National and State Services pages.

Other

Further information on Castleman Disease can be found at:

References

National Organization for Rare Diseases. Castleman Disease. Published 1988-2022. Updated 16 June 2022. Accessed 21 August 2024. https://rarediseases.org/rare-diseases/castlemans-disease
Castleman Disease Collaborative Network. Accessed 10 August 2024. https://cdcn.org/castleman-disease/
Orphanet. Castleman disease. Accessed 20 August 2024. https://www.orpha.net/en/disease/detail/160
Lomas OC, Streetly M, Pratt G, Cavet J, Royston D, Schey S, et al. The management of Castleman disease. British Journal of Haematology. 2021;195(3):328-37. https://doi.org/10.1111/bjh.17688
van Rhee F, Oksenhendler E, Srkalovic G, Voorhees P, Lim M, Dispenzieri A, et al. International evidence-based consensus diagnostic and treatment guidelines for unicentric Castleman disease. Blood Advances. 2020;4(23):6039-50.
Carbone A, Borok M, Damania B, et al. Castleman disease. Nature Reviews Disease Primers. 2021;7(1):84. https://doi.org/10.1038/s41572-021-00317-7.
Fajgenbaum DC, Uldrick TS, Bagg A, et al. International, evidence-based consensus diagnostic criteria for HHV-8–negative/idiopathic multicentric Castleman disease. Blood. 2017;129(12):1646-57. https://doi.org/10.1182/blood-2016-10-746933
Genetic and Rare Disease Information Centre. Castleman Disease. Updated July 2024. Accessed 21 August 2024. https://rarediseases.info.nih.gov/diseases/12656/castleman-disease

Page Last Updated

26/09/2024 01:30

Diseases of the immune system