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.
Hiding in Plain Sight: Could This Be Castleman Disease?
Hiding in Plain Sight: Could This Be Castleman Disease?
Welcome to CME on ReachMD. This episode is part of our MinuteCME curriculum.
Prior to beginning the activity, please be sure to review the faculty and commercial support disclosure statements as well as the learning objectives.
This is CME on ReachMD, and I'm Dr. Corey Casper. Here with me today is Dr. Sudipto Mukherjee and Jovanna, a patient who has been diagnosed with Castleman’s disease.
Let's dive right in with a discussion of the clinical burden of Castleman’s disease and the impact that a delayed diagnosis has on our patients. So starting with Dr. Mukherjee, what are some of the challenges in identifying and diagnosing patients with Castleman’s disease?
Castleman disease patients can have myriad presentations, depending upon the particular type of Castleman’s disease and the extent of underlying inflammation. And that determines how much of a clinical burden each patient will experience through their clinical journey.
For example, for patients with idiopathic multicentric Castleman disease, or iMCD, the focus of this discussion, presentation can vary widely, ranging from a mild flu-like illness, to commonly seen constellation of findings such as fevers, night sweats, pain, lymphadenopathy at multiple sites, enlarged liver or spleen, generalized fluid accumulation in the body or in the belly, to severe sepsis-like picture with multi-organ failure and death. These symptoms can present abruptly or over a period of time with multiple hospitalizations and multiple specialty visits in the interim.
Diagnosis is very challenging as it is a multi-step process that requires excisional biopsy of the involved lymph nodes, demonstrating the specific histopathologic features, meeting certain lab criteria and clinical criteria, and importantly, exclusion of a variety of conditions that mimics iMCD.
Two of the most frequently encountered challenges in diagnosis is reliance on fine-needle aspiration biopsy of the involved lymph nodes for histopathological examination and some level of lack of awareness of the diagnostic criteria. But early diagnosis is critical as it leads to timely initiation of IL-6-directed therapy.
Now, I will turn over to Jovanna so that she can share with us her experience leading up to her diagnosis of Castleman’s disease.
Thanks, Doctor. So my first symptom was an incredible pain that I had in my abdomen, and that led to a myriad of other symptoms popping up as I got more and more sick. I had horrible night sweats. I was sweating through my clothes and my sheets every single night. My body began to swell and my abdomen got so swollen and full of fluid that I looked like I was 9 months pregnant. I was always nauseous or vomiting. And on my third hospital visit, they found out that my kidneys were functioning at 14%. By that time, I was so frustrated with going in and out of the hospital with no answers that I was fighting to go home. I ended up on dialysis and a PCA [patient-controlled analgesia] pump that gave me pain medication every 8 minutes because I wasn't able to function by myself because I was in such incredible pain. I cried all the time, because the pain was getting worse and worse, and no one had answers for me. At first, most of my doctors thought that I had lymphoma because of all the lymph nodes that I had all over my body that were an increased size, but nothing else checked the boxes of lymphoma, so it became kind of an infuriating puzzle for everyone, including myself.
Thank you for sharing your story with us, Jovanna. That sounds incredibly difficult and frustrating, and I can only imagine how frightening that must have been for you.
You know, Jovanna and Dr. Mukherjee both, I think, provided us with a very consistent and, I think, all too common portrait of what Castleman’s disease looks like in a patient when it first presents. From Jovanna, we heard that there are a wide variety of symptoms and these include pain, night sweats, swelling, nausea and vomiting, and organ dysfunction. And again, the experience that Jovanna had with multiple attempts – 3 hospitalizations before a diagnosis was reached, is very common in patients with Castleman’s disease.
From Dr. Mukherjee, again, we learned that it's this myriad of symptoms and the multistep aspect of how to make definitive diagnosis, the lack of familiarity that clinicians have with the guidelines around making a diagnosis all lead to delayed diagnoses.
So this has been a great initial bite-sized discussion. Unfortunately, our time is up for now. Thank you all for listening, and I look forward to exploring this further in subsequent sessions.
You have been listening to CME on ReachMD. This activity is provided by Prova Education and is part of our MinuteCME curriculum.
To receive your free CME credit, or to download this activity, go to ReachMD.com/Prova. Thank you for listening.
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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