Diseases of the Immune System
Idiopathic Multicentric Castleman Disease (iMCD)
Rare Disease Classifications:
ORPHA:570431 Idiopathic Multicentric 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.
Quick Page Search:
Emergency Management | Clinical Care Guidelines | Synonyms | Summary | Personal Stories | Symptoms | Cause/Inheritance | Diagnosis | Treatment | Clinical Care | Research and Data | Rare Disease Organisation(s) | Support Services/Resources | Mental Health | Other | References
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 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
HHV-8-negative multicentric Castleman disease, HHV8-MCD, Human herpesvirus-8-negative multicentric Castleman disease, Castleman Disease, CD, multicentric Castleman disease, MCD, angiofollicular lymph node hyperplasia, angiomatous lymphoid, angiofollicular ganglionic hyperplasia, Castleman tumour, giant benign lymphoma, giant lymph node hyperplasia, hamartoma of the lymphatics
Summary
Idiopathic multicentric Castleman disease (iMCD) is a type of Castleman disease where multiple lymph nodes throughout the body become enlarged and overactive, for an unknown reason.1-3 Lymph nodes are part of the immune system and are the home base of immune cells. There are hundreds of lymph nodes in the human body. The overactive lymph nodes result in an overproduction of immune cells which can cause inflammation and damage tissues and organs throughout the body.
iMCD can be split into two subtypes depending on the symptoms present:2,4,5
- Idiopathic multicentric Castleman disease-TAFRO (iMCD–TAFRO) – characterised by the presence of thrombocytopenia (unusually low platelet count), anasarca (swelling of multiple tissues or organs), fever, reticulin fibrosis or myelofibrosis (bone marrow disease or cancer) and organomegaly (enlargement of any organs)
- Idiopathic multicentric Castleman disease-not otherwise specified (iMCD-NOS) – individuals typically have less severe symptoms and complications compared to iMCD-TAFRO
Below are other types of Castleman disease which have some overlapping symptoms with iMCD but different complications, causes, and treatment methods:
- Unicentric Castleman disease (UCD) – only involves one or one group of lymph nodes
- HHV-8-associated multicentric Castleman disease (HHV-8+MCD) – affects multiple lymph nodes, similar to iMCD but is 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)
Useful Links for Professionals
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 signs of idiopathic multicentric Castleman disease (iMCD) can have a slow and subtle onset or be sudden and life threatening. The most common symptoms of iMCD include:1,2,6,7
- enlarged lymph nodes/lumps under the skin in more than one place
- flu-like symptoms which may include aches, pains, tiredness, night sweats, weight loss, appetite loss, or nausea
- spotted red or purple skin rash (cherry hemangioma or violaceous papules)
- swelling or enlargement of any part of the abdomen or organs (oedema/ascites)
- tingling or numbness in the hands or feet (peripheral neuropathy)
- difficulty breathing
Idiopathic multicentric Castleman disease can progress to multi-system organ failure and involve varying symptoms related to the specific organs impacted.1,2,6,7
There are two subtypes of idiopathic multicentric Castleman disease:
Idiopathic multicentric Castleman disease-TAFRO (iMDC–TAFRO)
Some people with iMCD also experience acute episodes of some of the following 5 symptoms: thrombocytopenia (unusually low platelet count), anasarca (swelling of multiple tissues or organs), fever, reticulin fibrosis or myelofibrosis (bone marrow disease or cancer) and organomegaly (enlargement of any organs). 2,5 This subtype generally has more severe complications than iMCD without TAFRO.
Idiopathic multicentric Castleman disease-not otherwise specified (iMCD-NOS)
This includes all individuals with iMCD without TAFRO. The disease generally involves less severe symptoms.2,4 A subset of individuals with iMCD-NOS have chronic thrombocytosis (high levels of platelets) and hypergammaglobulinemia (high levels of antibodies). This is sometimes refered to as idiopatic multicentric Castlman disease with plasmacytic lymphadenopathy (iMCD-IPL).6,7
Please speak to your medical team to learn more about the symptoms and complications of the different subtypes of iMCD.
Cause/Inheritance
The cause of idiopathic multicentric Castleman disease (iMCD) is unknown.1 There are no known risk factors.
Diagnosis
Diagnosis of Idiopathic multicentric Castleman disease (iMCD) may be made based on the results of an excisional lymph node biopsy and blood and urine tests.4,9 An excisional lymph node biopsy involves the removal of an entire lymph node (either under local anaesthetic while you are awake or general aesthetic while you are asleep). The lymph node is then examined under a microscope to look for characteristic signs of Castleman disease.
Blood and urine tests may also be performed look for other signs of iMCD such as:4,5,8
- anaemia (low amount of red blood cells)
- high inflammatory markers in blood (such as CRP and/or ESR)
- protein and blood cells in urine which can indicate kidney disease
- high number of platelets (clotting agent in blood)
- high globulins and low albumin levels in blood
- high levels of plasma cells in bone marrow and lymph nodes
Differential diagnosis (to rule out other conditions) includes unicentric Castleman disease, KSHV/HVV-8-associated Castleman disease, POEMS syndrome, Hodgkin lymphoma, non-Hodgkin lymphoma, Systemic lupus erythematous, Rheumatoid arthritis and other autoimmune diseases, and acute viral infections including EBV and HIV infection.2,4,5,8-10
Treatment
There is no curative treatment for idiopathic multicentric Castleman disease (iMCD) but there are medications to manage the symptoms.4,5,8 These include biologic medications which can reduce inflammation. In some cases where the symptoms are severe and/or don’t respond to biologic medications, corticosteroids and/or chemotherapy may be used. Other types of medications may also be used to treat complications on a case-by-case basis.
It is best to speak with your medical team to learn more about possible treatments for Castleman disease and to determine suitability of treatment. Treatment will depend on an individual’s specific symptoms and complications.
Clinical Care
Healthcare professionals involved in the treatment of Castleman disease may include general practitioners (GP), paediatricians, haematologists, pulmonologist, nephrologists and oncologists.2,8,11 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.
Support Services/Resources
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:
- RARE Portal: Unicentric Castleman Disease (UCD)
- Genetic and Rare Diseases (GARD) Information Center: Castleman Disease
- National Organization for Rare Disorders (NORD): Castleman Disease
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
- Gao Y-h, Liu Y-t, Zhang M-y, et al. Idiopathic multicentric Castleman disease (iMCD)-idiopathic plasmacytic lymphadenopathy: A distinct subtype of iMCD-not otherwise specified with different clinical features and better survival. British Journal of Haematology. 2024;204(5):1830-7. https://doi.org/10.1111/bjh.19334
- Nishikori A, Nishimura MF, Nishimura Y, et al. Idiopathic Plasmacytic Lymphadenopathy Forms an Independent Subtype of Idiopathic Multicentric Castleman Disease. Int J Mol Sci. 2022;23(18). https://doi.org/10.3390/ijms231810301
- 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. https://doi.org/10.1182/bloodadvances.2020003334
- 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/
- 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 15:25