Castleman's Disease
What is Castleman's Disease?
Castleman's Disease is a rare disorder characterized by abnormal non-cancerous growths in lymph node tissue. The lymphatic system moves fluid and distributes white blood cells to tissues to help prevent infection. Lymph nodes filter out invading substances in the lymph, such as viruses, bacteria, and cancer cells. There are many lymph nodes found throughout the body in the neck, armpit, chest, abdomen, and groin. There are two main types of Castleman's Disease depending on the number of lymph nodes affected:
1. Unicentric Castleman's Disease (UCD): This form affects a single lymph node or region of lymph nodes.
2. Multicentric Castleman's Disease (MCD): This form involves multiple lymph node regions and can affect other organs. It is further divided into idiopathic MCD (iMCD) and HHV-8-associated MCD (caused by human herpesvirus 8).
Synonyms
- Angiofollicular lymph node hyperplasia
Castleman's Disease is a rare disorder characterized by abnormal non-cancerous growths in lymph node tissue. The lymphatic system moves fluid and distributes white blood cells to tissues to help prevent infection. Lymph nodes filter out invading substances in the lymph, such as viruses, bacteria, and cancer cells. There are many lymph nodes found throughout the body in the neck, armpit, chest, abdomen, and groin. There are two main types of Castleman's Disease depending on the number of lymph nodes affected:
1. Unicentric Castleman's Disease (UCD): This form affects a single lymph node or region of lymph nodes.
2. Multicentric Castleman's Disease (MCD): This form involves multiple lymph node regions and can affect other organs. It is further divided into idiopathic MCD (iMCD) and HHV-8-associated MCD (caused by human herpesvirus 8).
Castleman's disease is considered to be very rare, with an overall estimated incidence of 21-25 cases per million person-years. UCD is more common than MCD.
| Name | Abbreviation |
|---|---|
| Angiofollicular lymph node hyperplasia |
The exact cause is unknown in many cases. Immune system dysregulation, associated with an overproduction of the immune-response regulator interleukin 6 (IL-6), may play a role. Infections with human herpesvirus 8 (HHV-8), particularly in those previously infected by human immunodeficiency virus (HIV), is believed to cause about 25-50% of MCD cases. The HHV-8 viral infection may induce further IL-6 production, leading to rapid cellular growth in lymph nodes.
Symptoms of Castleman’s Disease can vary based on whether it is UCD or MCD.
-
Unicentric Castleman's Disease (UCD):
-
Enlarged lymph node or mass, typically in the chest, abdomen, or neck
-
May be asymptomatic or cause pressure-related symptoms depending on the location of the mass
-
-
Multicentric Castleman's Disease (MCD):
-
Enlarged lymph nodes in multiple regions
-
Fever and night sweats
-
Unintended weight loss
-
Fatigue and weakness
-
Anemia (low red blood cell count)
-
Enlarged liver or spleen
-
Fluid buildup (edema)
-
Neurological symptoms in severe cases
-
Diagnosis of Castleman's Disease typically involves a combination of clinical evaluation, imaging studies, and laboratory tests:
-
Medical History and Physical Examination: A thorough examination to check for enlarged lymph nodes and other symptoms.
-
Imaging Studies: CT scans, MRI, or PET scans to identify enlarged lymph nodes and other affected areas.
-
Lymph Node Biopsy: The definitive diagnosis is made through a biopsy of the affected lymph node, which shows characteristic histopathological features.
-
Blood Tests: These can reveal anemia, elevated inflammatory markers (like C-reactive protein and erythrocyte sedimentation rate), and abnormalities in liver and kidney function.
HHV-8 Testing: For patients suspected of having MCD, especially those who are HIV-positive, testing for HHV-8 can be important.
-
Unicentric Castleman's Disease:
-
Surgical Removal: The primary treatment is the surgical removal of the affected lymph node.
-
Radiation Therapy: This may be used if surgery is not feasible.
-
-
Multicentric Castleman's Disease:
-
Anti-Viral Therapy: Anti-viral drugs such as Rittuximab for HHV-8-associated MCD.
-
Immunotherapy: IL-6 inhibitors such as siltuximab or tocilizumab can be effective.
-
Chemotherapy: Used in severe cases or when other treatments fail.
-
Corticosteroids: To manage inflammation.
-
Anti-Retroviral Therapy: For those with HIV-associated MCD.
-
Unicentric Castleman's Disease: Generally has an excellent prognosis after the surgical removal of the affected lymph node, with a high rate of complete remission.
- Multicentric Castleman's Disease: The prognosis can be more variable and depends on several factors, including the patient's overall health, the response to treatment, and whether there is an underlying condition such as HIV. MCD can be life-threatening if not properly managed, but with appropriate treatment, many patients can achieve remission or long-term control of the disease.
-
Castleman Disease Collaborative Network (CDCN): CDCN.
- NORD: https://rarediseases.org/rare-diseases/castlemans-disease/
Last updated 12 Oct 2016, 06:55 PM
12 Oct 2016, 06:55 PM
Patient story and scientific information on Castleman's in Science Magazine, Vol. 353, Pages 212-215, 2016.
Last updated 8 Jan 2014, 12:58 AM
7 Jan 2014, 11:42 PM
Mine was diagnosed when they removed the entire tumour, I think thats the only way to diagnose, before that, they thought it was a nerve sheath tumour, they ruled that out and then thought it was lymphoma, right up until my results came back. Have you spoken to Jim johnstone? he can put you in touch with any experts in your area or nearest, they can diagnose you if your current place cannot. Do you use FB? this is pretty active International Castlemans Disease Organization https://www.facebook.com/groups/48343887930/ also https://www.facebook.com/care4castlemans and the web page which Jim runs, he is very active on FB http://castlemans.org/
7 Jan 2014, 11:06 PM
I really think I do, yet the Dr.'s haven't said it in cement yet. I have very large lymph nodes in my chest, on my neck and jawline and my immune system is wiped out. I'm waiting for results on recent blood work. The dr.'s are finding me very difficult to diagnose. It's worrisome. Hope I don't get lymphoma. Thanks for answering. I had a media stenoscopy last March where they took many samples of the lymph nodes in the chest. It was inconclusive. In September I had a lung biopsy, since I also have a rare lung disease and that's when Castleman's was brought up. They just aren't willing to or have enough proof that that's what it is yet.
| Title | Date | Link |
|---|---|---|
| This Penn Professor Almost Died From a Rare Disease Four Times | 09/01/2019 | |
| This med student was given last rites before finding a treatment that saved his life. His method could help millions | 09/15/2019 | |
| Inspirational boy battling rare disease returns from hospital after 'life-saving' surgery | 04/19/2020 | |
| A patient-powered registry boosts the study of rare Castleman disease | 01/31/2021 | |
| Castleman Disease: Types, Symptoms, and Treatment | 05/22/2022 | |
| Rare Disease: Could Existing Drugs Turn the Tide? | 05/21/2023 | |
| Life saved: AI discovers existing drug works for rare disease | 07/15/2023 | |
| His Rare Disease's Cure Was Sitting on the Pharmacy Shelf | 11/04/2023 |
Clinical Trials
CoRDS, or the Coordination of Rare Diseases at Sanford, is based at Sanford Research in Sioux Falls, South Dakota. It provides researchers with a centralized, international patient registry for all rare diseases. This program allows patients and researchers to connect as easily as possible to help advance treatments and cures for rare diseases. The CoRDS team works with patient advocacy groups, individuals and researchers to help in the advancement of research in over 7,000 rare diseases. The registry is free for patients to enroll and researchers to access.
Enrolling is easy.
- Complete the screening form.
- Review the informed consent.
- Answer the permission and data sharing questions.
After these steps, the enrollment process is complete. All other questions are voluntary. However, these questions are important to patients and their families to create awareness as well as to researchers to study rare diseases. This is why we ask our participants to update their information annually or anytime changes to their information occur.
Researchers can contact CoRDS to determine if the registry contains participants with the rare disease they are researching. If the researcher determines there is a sufficient number of participants or data on the rare disease of interest within the registry, the researcher can apply for access. Upon approval from the CoRDS Scientific Advisory Board, CoRDS staff will reach out to participants on behalf of the researcher. It is then up to the participant to determine if they would like to join the study.
Visit sanfordresearch.org/CoRDS to enroll.
Community Leaders
Expert Questions
Ask a question
Community User List
Last year I was diagnosed with Castleman Disease. I am 27 years old, I was the third case in my country and the youngest one. I was lucky, I had a singular nodule in my left lung and with...
Start a Community
Don't See Your Condition On Rareshare?
Start your own! With a worldwide network of 8,000 users, you won't be the only member of your community for long.
FAQ
Have questions about rareshare?
Visit our Frequently Asked Questions page to find the answers to some of the most commonly asked questions.
Discussion Forum
Science Magazine article on Castleman's
Created by graychan | Last updated 12 Oct 2016, 06:55 PM
Castlemans Disease
Created by samoyed1 | Last updated 8 Jan 2014, 12:58 AM
Our Communities
Join Rareshare to meet other people that have been touched by rare diseases. Learn, engage, and grow with our communities.
FIND YOUR COMMUNITY
Our Resources
Our rare disease resources include e-books and podcasts
Our Community Leaders
Community leaders are active users that have been touched by the rare disease that they are a part of. Not only are they there to help facilitate conversations and provide new information that is relevant for the group, but they are there for you and to let you know you have a support system on Rareshare.