The 2016 "Beating Castleman's, Together" Patient & Loved Ones Summit
By CDCN Volunteer Alisa McDonald
The CDCN Patient Summit is an opportunity for patients to gather with others affected by Castleman disease. At the 3rd Annual Patient Summit in Philadelphia, PA, fifty patients and their loved ones from all over the United States as well as Germany, Japan and Australia were in attendance. The patients were given a unique chance to share their stories in a supportive forum, and to participate in activities aimed at furthering awareness and research. This gathering – the only one of its kind happening for Castleman disease patients – gives participants the opportunity to learn the basics about this rare disease, what resources are available, including treatment options and emotional support, and to learn about the future direction for Castleman disease research. The Patient Summit presenters, including researchers, clinicians and CDCN volunteers, updated patients and their loved ones on the research advancements made since the establishment of the CDCN in 2012.
The 3rd Annual Patient Summit on November 4, 2016 began with an overview of Castleman disease, explaining that it is the result of a hyperactive immune system. (Watch here: https://youtu.be/PTkhn5UfNX8). All people have cytokines (or proteins) as part of a healthy immune system, but in Castleman disease, specific cytokines may be present at very high levels. Dr. David Fajgenbaum, Executive Director of the CDCN and Castleman disease patient, explained the difference between a healthy immune response and a Castleman disease immune response using an analogy of a fire and firefighters. Just like when there is a fire and firefighters are dispatched, the immune system sends certain cells to eradicate a pathogen or infection. Once a healthy immune system destroys the pathogen, the immune system returns to a “monitoring or surveillance” state. In Castleman disease, the immune system does not subside and instead continues to launch an assault on a perceived pathogen. Now imagine a firehouse being alerted to fire, but having no idea where the fire is. There’s no way to find the fire, or put it out! In some Castleman patients, there is no “fire” that the immune system can find; there is no infection or foreign intruder making the person sick. So, the patient’s immune system goes into overdrive and subsequently attacks and destroys the patient’s healthy cells, tissues and organs.
All Castleman disease patients have lymph nodes with the same histopathological characteristics, but after that, it gets tricky. In Unicentric Castleman disease (UCD), the patient usually has one region of swollen lymph nodes due to an unknown trigger, but the lymph nodes can be removed with a good outcome for most patients. In HHV 8-positive multicentric Castleman disease (MCD), multiple regions of enlarged lymph nodes due to an HHV 8 infection. The most complicated form of Castleman disease is HHV 8-negative or "idiopathic" MCD, which has multiple regions of swollen lymph nodes, however, there’s no virus or known cause. There is no perceivable fire…
CDCN research is looking for the fire… Castleman disease is such an uncommon and enigmatic disease, so even the experts don’t have answers. What triggers the immune system to activate? Which immune cells are responsible? What is the active cellular pathway? CDCN research is trying to answer all these questions. Some supported research is already producing promising findings and speakers at the Patient Summit, including Dr. Fajgenbaum, Dr. Frits van Rhee and Dr. Jason Ruth, shared these advances in scientific understanding of this rare disease with patients and their loved ones. Watch these lectures here on the CDCN YouTube channel.
A common theme touched on throughout the Patient Summit is the CDCN’s novel approach to conducting research. Like many disease advocacy and health awareness groups, the CDCN fundraises to support research studies. Instead of awarding funding to researchers so they can conduct their own, independent studies, the CDCN identifies top research priorities via its network of physicians, researchers and patients and then finds the best researchers to perform these studies. The CDCN has direct oversight of all CDCN funded studies, from prioritizing the research ideas, designing the studies, fundraising and then conducting the studies. The CDCN is able to use this approach due to its strong and global network of patients, physicians and researchers, volunteers and donors committed to accelerating research of Castleman disease.
For patients wishing to contribute to the fight to beat Castleman disease, consider joining the ACCELERATE patient registry study and donating samples to the biobank. Castlebank will advance important research by consolidating samples, and making them accessible to researchers. Signing up for the ACCELERATE registry requires patients to initially fill out a survey, and consent to sharing their medical records and providing bio-specimen. After that, patients can follow-up with contact information and health updates every 6 months to keep their records up-to-date. Donating samples to the biobank is a significant way patients can work toward research breakthroughs for a disease few people outside of the CDCN are aware. One CDCN volunteer called patients to sign-up for Castlebank and ACCELERATE to advance research in a way only they can, “You have the ability not only to help the fight, you have the ability to be the fight!”
So will you join us in the fight?
- Munshi, Nikhil, et al. "Use of a claims database to characterize and estimate the incidence rate for Castleman disease." Leukemia & lymphoma 56.5 (2015): 1252-1260.
- Liu, A. Y., Nabel, C. S., Finkelman, B. S., Ruth, J. R., Kurzrock, R., van Rhee, F., ... & Fajgenbaum, D. C. (2016). Idiopathic multicentric Castleman's disease: a systematic literature review. The Lancet Haematology, 3(4), e163-e175.