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)

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.^1,3

Universal rare disease classifications provide a common language for recording, reporting and monitoring diseases. Please visit the Rare Disease Classifications page for more information about these internationally recognised classifications.

ORPHA:160 Castleman Disease

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

The types of Castleman disease have different symptoms and complications. Please refer to the individual Castleman disease pages on the RARE Portal for more information:

• Unicentric Castleman Disease (UCD)
• Idiopathic multicentric Castleman disease (iMCD)
• HHV-8-associated multicentric Castleman Disease (HHV-8+MCD)

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

Rare diseases are often serious and progressive, exhibiting a high degree of symptom complexity, leading to significant disability. Majority of the estimated two million Australians living with a rare disease meet the Australian Government’s definition for disability (in accordance to the Australian Public Service Commission and Australian Bureau of Statistics), and many experience severe and permanent disability impacts. If you or someone you care for is experiencing disability-related impacts from a rare condition, please speak with a health or disability professional for advice. Information about relevant disability support can be found at the RARE Portal’s Disability Support Information page.

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.

HHV-8-associated multicentric Castleman disease (HHV-8+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

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.

To differentiate multicentric Castleman disease (multiple lymph nodes affected) from unicentric Castleman disease (singular lymph node affected), a computerised tomography (CT) or positron emission tomography–computed tomography (PET-CT) scan is usually done to look for the presence of other affected lymph nodes.^5,6

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:

• Unicentric Castleman Disease (UCD)
• Idiopathic multicentric Castleman disease (iMCD)
• HHV-8-associated multicentric Castleman Disease (HHV-8+MCD)

A differential diagnosis can rule out other conditions with similar symptoms, such as 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 a particular type of Castleman disease.

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:

• Unicentric Castleman Disease (UCD)
• Idiopathic Multicentric Castleman Disease (iMCD)
• HHV-8-associated multicentric Castleman Disease (HHV-8+MCD)

Please speak to your medical team to learn more about the possible treatment or management options for your condition. Treatment will depend on an individual’s specific condition and symptoms. It is also important to stay connected to your medical team so that you can be made aware of any upcoming clinical trial opportunities.

Clinical care for rare diseases often involves a multidisciplinary team of medical, care and support professionals. Please note that the information provided here is as a guide and that RVA does not necessarily monitor or endorse specific clinics or health experts.

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.

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.”

Individuals living with rare diseases may have complex medical issues and disabilities, which are not always visible. It is often useful to refer to their medical history as well as personal information such as a medical card, doctor’s letter, or if available, a rare disease passport, for relevant information.

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

There are specific considerations around participating in rare disease research, including clinical trials. It is important to be mindful of issues such as data privacy, research ethics, consent and differences in research regulations between Australia and other countries.

If you are interested in finding clinical trials for your condition, please visit the following websites; however, there may not be any clinical trials available:

• Australian Clinical Trials
• ClinicalTrials.gov

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

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

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. If you are interested in starting an Australian organisation, please see Engaged, Ethical and Effective: A Guide for Rare Disease Organisation Leaders in Australia.

Please note that RVA does not monitor or endorse each group/organisation’s operational governance and activities. When engaging with a group, please consider the information on the RARE Portal’s Finding Helpful Peer and Community Supports page.

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

If you would like to share your personal story with RVA, please visit the Rare Voices Australia: Share Your Story page. RVA will consider your story for publishing on our website and inclusion on the RARE Portal.

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.

People living with a rare disease, including families and carers, often face unique challenges such as diagnostic delays, misdiagnoses, limited treatment options, and limited access to rare disease specialists and support. These challenges may impact people’s emotional wellbeing and quality of life. Many people find it helpful to seek mental health and wellbeing support to cope with ongoing stress and uncertainty. Connecting with people who have shared experiences through a support group may also be helpful. Information about relevant mental health and wellbeing support can be found at:

• Mental Health and Wellbeing Support for Australians Living with a Rare Disease
• The National and State Services pages underneath the ‘Mental Health’ sections listed

Further information on Castleman disease can be found at:

• Genetic and Rare Diseases (GARD) Information Center: Castleman Disease
• National Organization for Rare Disorders (NORD): Castleman Disease

• Orphanet: Castleman Disease

This page has been developed by Rare Voices Australia (RVA)’s RARE Portal team.

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.