Patient-Funded Research Reveals New Info about CD

6/20/2018 The Castleman Disease Collaborative Network (CDCN) is proud to announce the publication of our SPEED I (Serum Proteomics Evaluation for Etiology and Pathogenesis Data I) research study in the American Journal of Hematology in April 2018 titled Plasma proteomics identifies a ‘‘chemokine storm’’ in idiopathic multicentric Castleman disease. The study found that iMCD is not simply a disorder of IL-6;  it’s a complex inflammatory disorder that the global medical community is just beginning to unravel.

This publication (full text here), which would have not been possible without the funding support from our patient community, represents a major stride for the CDCN towards greater understanding of Castleman disease (CD) biology. The CDCN would like to recognize and thank the family and friends of Raj Jayanthan, a Castleman disease patient and Warrior, for generously funding this study.

Key Takeaways

There are several key take-aways from the study, which measured 1,129 molecules in 13 blood samples from 6 patients with the most deadly subtype of CD, idiopathic multicentric CD (iMCD).

1) Chemokines and cytokines were the groups of molecules most up-regulated and down-regulated in patients’ blood during flare compared to remission. These chemokines and cytokines represent immune system activation and promote inflammation.

2) iMCD is not simply a disease of excess interleukin-6 (IL-6); there were other cytokines such as CXCL13 even more up-regulated than IL-6.

3) CXCL13 plays an important role in lymph node development and immune system functioning, which are both abnormal in iMCD.

4) The levels of the different molecules were able to distinguish the cases with the newly described subtype of iMCD called TAFRO (thrombocytopenia, anasarca, fibrosis of the bone marrow, renal dysfunction, and organomegaly) syndrome from the other cases.

5) Molecules associated with the PI3K/Akt/mTOR signaling pathway, which is involved in cellular proliferation and immune system activation, were over-represented among the most up-regulated and down-regulated.

These findings may help to identify new tests for diagnosing iMCD, new treatments, and new guidelines for caring for the different subtypes of iMCD. However, future studies are needed with additional patients to build upon this knowledge base. Sheila Pierson, MS, the lead author on this study commented:

“We’re very excited to have published the first unbiased, systematic quantification of plasma proteins in iMCD!  We have known that iMCD clinical presentation can be very heterogeneous, and with this study, we now also demonstrate iMCD proteomic heterogeneity.  We’ve identified molecules not previously known to be associated with iMCD, and we believe that those findings are an important contribution to the field. More importantly, we’re actively pursuing further research based those findings.  Lastly, it’s extremely rewarding to have completed this study, which was funded entirely by patient support.”

Thank you to Castleman Warrior, Raj Jayanthan! 

We would like to recognize and thank the friends and family of Raj Jayanthan, a Castleman disease patient, for generously funding this study. Raj raised the funds through an online donation page through the Castleman Warrior program. When asked about what it meant to fund research that may have an impact on the lives of patients with Castleman disease, like himself, Raj shared the following message:

I’m incredibly grateful to the CDCN for providing an outlet for me to help fight back against this disease. During the months in recovery following my hospitalization, many of my friends and family asked me for ways that they could help. Before the CDCN, I initially didn’t have an answer for them. But when I discovered the CDCN and how it was leading the fight against this disease by mobilizing the scientific community to collaborate on Castleman disease research all while supporting patients and their loved ones along the way, I knew that we needed to do our part. I am extremely humbled by the outpouring of generosity by my friends and family on my behalf and so proud that we were able to fund such an important study. To everyone that has supported us these past few years and to everyone planning to support us in the future, thank you!!”

After helping to enable this study, Raj became more and more involved with the CDCN. He is now the CDCN’s Chief Patient Officer, which he does as a volunteer while completing his pediatrics residency training.

Final Thoughts from Dr. Fajgenbaum

The CDCN’s co-Founder & Executive Director, Dr. David Fajgenbaum, who served as the senior investigator on this study, shared the following quote:

“SPEED I represents exactly what we envisioned for the CDCN: patient-powered, expert guided, high-impact research that will be disseminated worldwide. I am so grateful for the hard work that so many people invested.  The CDCN’s global network conceptualized and prioritized this study. Raj and his family raised the funds to make this possible. Frits van Rhee and Katie Stone from the University of Arkansas for Medical Sciences contributed samples and expertise. Sheila Pierson led the execution of the project, including the multi-dimensional bioinformatic analyses.”

You can check out the CDCN Research Pipeline for a complete listing of the CDCN’s research studies.

Email for questions about the study.

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