Castleman Disease (CD)

Diseases of the immune system

Rare Disease Classifications:

ORPHA:160 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
International, evidence-based consensus diagnostic criteria for HHV-8–negative/idiopathic multicentric Castleman disease(iMCD); published in 2016 by a working group of 34 experts in iMCD and related disorders from 8 countries (including 2 physicians that are also iMCD patients)
International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease (iMCD) was established in 2018 by a working group of 42 experts in iMCD and related disorders from 10 countries;
- the effectiveness of the practices recommended in these guidelines has since been evaluated in a trial and reported in a 2023 publication, “Treatment consistent with idiopathic multicentric Castleman disease guidelines is associated with improved outcomes.”

Synonyms

CD, UCD, MCD, iMCD, POEMS-MCD, Angiofollicular lymph node hyperplasia, Angiomatous lymphoid, Angiofollicular ganglionic hyperplasia, Castleman tumour, Giant benign lymphoma, Giant lymph node hyperplasia, Hamartoma of the lymphatics

Summary

Castleman disease is a condition that is characterised by presence of enlarged lymph nodes with specific changes that can only be seen under a microscope.^1-3 Lymph nodes are tissues that act as the home base of immune cells. There are hundreds of lymph nodes in the human body. In Castleman disease the immune cells become over activated and can release excessive signals that can damage the body.

Castleman disease is categorised into different types, based on the number and location of the lymph nodes involved. They have different symptoms, causes, and treatments. The first signs of Castleman disease can either have a slow and subtle onset or be sudden and life threatening.

The two main types of Castleman disease are:^1-5

Unicentric Castleman disease (UCD) – only involves one or one group of lymph nodes
Multicentric Castleman disease (MCD) – involves multiple nodes around the body; MCD can be further divided into the following subtypes:
- Idiopathic Multicentric Castleman Disease (iMCD)– the cause of the disease is unknown
- HHV-8-associated multicentric Castleman disease (HHV-8+MCD)– caused by Human herpesvirus 8 (HHV-8) infection
- Castleman disease can also occur as a part of POEMS syndrome and is known as POEMS-associated multicentric Castleman disease (POEMS-MCD)

Different types of Castleman Disease

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

The types of Castleman disease have different symptoms and complications, which can be found on the individual Castleman disease pages on the RARE Portal:

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

Cause/Inheritance

The cause of Unicentric Castleman disease (UCD) and the two idiopathic subtypes of multicentric Castleman disease (iMCD) are unknown.^1,2,5,6 There are also no known risk factors.

KSHV/HHV-8-associated multicentric Castleman disease (HHV8+MCD) is caused by Kaposi’s sarcoma-associated herpesvirus (KSHV), also known as Human herpesvirus 8 (HHV-8) infection.^1,4,5 Please visit the HHV-8-associated multicentric Castleman disease (HHV-8+MCD) page on the RARE Portal for more information.

Castleman disease can also occur as a part of POEMS syndrome.^4,6,7 Please visit the POEMS-associated Multicentric Castleman Disease (POEMS-MCD) page on the RARE Portal for more information.

Diagnosis

Diagnosis of Castleman Disease may be made based on the results of an excisional lymph node biopsy as well as blood and urine tests.^1,4,8 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.

Further information about the diagnostic methods for the different types of Castleman disease can be found on the individual Castleman disease pages on the RARE Portal:

Differential diagnosis (to rule out other conditions) includes Hodgkin lymphoma, non-Hodgkin lymphoma, Systemic lupus erythematous, Rheumatoid arthritis and other autoimmune diseases, POEMS syndrome, and acute viral infections including EBV and HIV infection.^{2, 4-8}

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

Treatment

Treatment or management strategies may vary depending on the type of Castleman disease. More information can be found on the individual Castleman disease pages on the RARE Portal:

Clinical Care

Healthcare professionals involved in the treatment of Castleman disease may include general practitioners (GP), paediatricians, haematologists, pulmonologist, nephrologists and oncologists.^2,6,9 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
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
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
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
National Organization for Rare Disorders. POEMS Syndrome. Published 1991-2021. Updated 23 September 2021. Accessed 22 August 2024. https://rarediseases.org/rare-diseases/poems-syndrome/
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. https://doi.org/10.1182/bloodadvances.2020003334
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 12:45

Diseases of the immune system