This series of bite-sized episodes will take you on the winding journey that patients with idiopathic multicentric Castleman disease (iMCD) often undergo to achieve an accurate diagnosis. Once the condition is finally identified, even the most astute specialists can find themselves challenged by the treatment, management, and monitoring of these patients. Join Drs. Corey Casper and Sudipto Mukherjee as they explain the ups and downs of this rare disease.
Optimizing Treatment and Management of Castleman
Optimizing Treatment and Management of Castleman
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Welcome to this CME on ReachMD, and I'm Dr. Sudipto Mukherjee. Here with me today is Dr. Corey Casper and Jovanna, a patient who has been diagnosed with Castleman disease.
Let's hear from Jovanna. Can you give us a brief overview of your experience with being treated for Castleman disease?
Yes, so getting my diagnosis was quite literally life changing. I remember my doctor coming in, giving me my full diagnosis, iMCD [idiopathic multicentric Castleman disease] with TAFRO. And they gave me a couple options for treatment. So they put me on steroids. They started me on an infusion called siltuximab, and they also started me on an infusion of rituximab. Unfortunately, they had to pause the rituximab because I had an adverse reaction, but I was able to stay on the steroids for a small period of time. And I continued on siltuximab and have continued siltuximab to this day. I have it every 6 weeks. And it really has been a life changer. I am able to do the things I used to do before I got sick. And I function just like a healthy person would for most the time. I still have chronic illness fits, but it really has been life changing to have my diagnosis.
Thank you very much for sharing your experience.
Dr. Casper, how do you choose the right treatment for patients like Jovanna? And once you select a treatment, how do you monitor and manage any potential adverse events from treatment?
Thanks, Dr. Mukherjee, and again thanks, Jovanna, for sharing your story. And it's so wonderful to hear that you have found a treatment that seems to control your disease.
So the treatment of idiopathic multicentric Castleman’s disease really relies on targeting the central driver, which is interleukin-6. So too much interleukin-6 is made, and the key treatment option for treating Castleman’s disease is to antagonize that interleukin-6 with an interleukin-6 inhibitor.
So there's a single FDA-approved medication for the treatment of idiopathic multicentric Castleman’s disease; that is siltuximab, an IL-6 inhibitor, and really that is the mainstay of treatment. So patients like Jovanna typically would be offered an interleukin-6 inhibitor like siltuximab as first-line therapy. The traditional way that this is prescribed is it’s given once every 3 weeks, really for the rest of the patient’s life.
Depending on the severity of the initial presentation of Castleman’s disease, many providers may elect to give concomitant corticosteroids. And those corticosteroids can reduce some of the inflammation, especially in severe disease. But most typically, you would not continue those corticosteroids for prolonged periods of time because of their side effects and because eventually they lack efficacy.
Once those patients have begun their treatment, you look to see whether they’ve responded to it. And typically, the way we monitor response to treatment for Castleman’s disease is with a series of indicators. There’s the clinical indicator. So first and foremost, if the patient isn’t feeling better on some of their subjective symptoms, like their night sweats or their fevers or their fatigue, then, really, what’s the point in continuing. So we look at some of the patient’s specific subjective symptoms. Then of course, there’s less subjective and more objective measures, laboratory responses. So typically, we’d like to see improvements in anemia, reductions in inflammation usually as evidenced by a C-reactive protein, and a return to a normalization of any organ dysfunction. And finally, we would look to see if there’s any radiographic improvements. So we would like to see Ideally the size of organs reduce and lymph nodes to shrink.
Adverse events with treatment for Castleman’s disease are rare. So with siltuximab, it is a monoclonal antibody against interleukin-6. And you can see allergy to this, a hypersensitivity. And that's typically treated by the way you would do this with other monoclonal products, by reducing the rate of infusion, by sometimes giving symptomatic treatment to that with diphenhydramine or steroids, and really to continue to monitor them.
Finally, this treatment is continued until or if there is disease progression. If there's disease progression beyond first-line therapy, this often is within the realm of a specialist. So at this point, most patients with Castleman’s disease would be referred to a specialty center where usually a combination of monoclonal antibodies but also conventional chemotherapy would be recommended.
Thank you, Dr. Casper, for your concise and comprehensive review of the clinical management of iMCD patients.
Well, this has been a brief but great discussion. Unfortunately, our time is up. Thanks for listening.
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In accordance with the ACCME Standards for Integrity and Independence, Global Learning Collaborative (GLC) requires that individuals in a position to control the content of an educational activity disclose all relevant financial relationships with any ineligible company. GLC mitigates all conflicts of interest to ensure independence, objectivity, balance, and scientific rigor in all its educational programs.
Corey Casper, MD, MPH
Clinical Professor of Medicine and Global Health
University of Washington
Affiliate Professor, Fred Hutch Cancer Center
Research: Amyris, ImmunityBio (IBRX), Janssen
Ownership Interest: Viracta Therapeutics
Receives Royalties: UpToDate
Patent Holder: AAHI
Consulting Fees: EUSA Pharma
Sudipto Mukherjee, MD, PhD, MPH
Director, Rare Cancers & Blood Diseases
Cleveland Clinic Taussig Cancer Institute
Research: BMS/Celgene, Jazz Pharma, Novartis
Receives Royalties: McGraw Hill
Consulting Fees: BioPharm, Blueprint Medicines, BMS/Celgene, Genentech and AbbVie, EUSA, Novartis
- Cindy Davidson has nothing to disclose.
- Stephen Chavez has nothing to disclose.
- Libby Lurwick has nothing to disclose.
- Colleen Resnick has nothing to disclose.
- Mara Siegel has nothing to disclose.
After participating in this educational activity, participants should be better able to:
- Discuss the clinical burden and impact associated with a delayed diagnosis of Castleman disease
- Improve the speed and accuracy of a diagnosis of Castleman disease through increased recognition of its clinical presentation and knowledge of published diagnostic criteria
- Incorporate guideline-concordant care into the treatment of patients with Castleman disease
- Employ shared decision-making strategies to increase patient participation and promote advocacy, clinical trial enrollment, and research
This activity is designed to meet the educational needs of US hematologist oncologists, rheumatologists, infectious disease specialists, immunologists, and other HCPs who are involved in diagnosing, managing, and treating iMCD.
In support of improving patient care, Global Learning Collaborative (GLC) is jointly accredited by the Accreditation Council for Continuing Medical Education (ACCME), the Accreditation Council for Pharmacy Education (ACPE), and the American Nurses Credentialing Center (ANCC) to provide continuing education for the healthcare team.
Global Learning Collaborative (GLC) designates this enduring activity for a maximum of 1.0 AMA PRA Category 1 Credits™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.
Global Learning Collaborative (GLC) designates this activity for 1.0 nursing contact hour. Nurses should claim only the credit commensurate with the extent of their participation in the activity.
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This activity is supported by an independent educational grant from Recordati.
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