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Treating patients with iMCD

  • About iMCD
  • Diagnosing iMCD
  • Treating iMCD
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Importance to Treat

Even after it’s diagnosed, iMCD often leads to hospitalization

60% of patients were hospitalized the first year after iMCD diagnosis
  • Idiopathic multicentric Castleman disease (iMCD) is a progressive disease with a poor prognosis2,3
  • Regardless of severity of clinical presentation, high rates of hospitalizations and ED visits are observed in patients with iMCD2

aRetrospective database claims analysis that assessed hospitalizations, ED visits, iMCD-related comorbidities, and organ failure in US patients with Castleman disease (over 30 million) between January 1, 2017, and December 31, 2019. Minor criteria from the Castleman Disease Collaborative Network (CDCN) diagnostic criteria were applied to ensure that the patients had iMCD.1,3

Despite diagnosis, most patients with iMCD are undertreated4

More than 70% of patients receive steroid monotherapy
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Treating patients with iMCD

There are various therapies being used in the treatment of patients with iMCD. The CDCN, the same group that published the first consensus diagnostic criteria, has developed formal guidelines for the treatment of iMCD.5

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Timely
Diagnosis

Timely diagnosis and appropriate treatment can help bring the symptoms of iMCD under control.3

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Ongoing
Treatment

Considering the chronic and progressive nature of iMCD, patients will need to receive ongoing treatment to keep their symptoms under control.5,6

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Following Treatment Guidelines

Evidence-based treatment guidelines have been developed to help healthcare professionals treat their patients with iMCD.5

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Learn more about an iMCD treatment that may be right for your patient

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Different journeys. Same fighting spirit.

Hear from the people who have been fighting iMCD for years, and learn how they have dealt with some of the challenges they have experienced.

Jessica

In 2005, Jessica began experiencing fever, night sweats, and swollen lymph nodes. After 14 long years, she found out that she has iMCD. Watch her story to learn about her journey to diagnosis.

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Carl

In 1985, there weren’t any guidelines to diagnose iMCD. But that didn’t stop Carl from getting an accurate diagnosis for his rare disease. Watch his journey to learn about the path he took to finally get diagnosed with iMCD.

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Frank & Dina

iMCD has troubled Frank for years. But with his wife Dina by his side, he was never going to back down. Watch the video to learn about the ups and downs they faced during their journey to diagnosis.

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References:
  1. Mukherjee S, Martin R, Sande B, et al. A longitudinal population level analysis of healthcare resource utilization, comorbidity, and survival in idiopathic multicentric Castleman disease patients. Presented at: 62nd American Society of Hematology (ASH) Annual Meeting and Exposition; December 5-8, 2020; virtual meeting.

  2. Mukherjee S, Martin R, Sande B, et al. A longitudinal population level analysis of healthcare resource utilization, comorbidity, and survival in idiopathic multicentric Castleman disease patients. Blood. 2020;136(suppl 1):11.

  3. Fajgenbaum DC, Uldrick TS, Bagg A, et al. International, evidence-based consensus diagnostic criteria for HHV-8–negative/idiopathic multicentric Castleman disease. Blood. 2017;129(12):1646-1657.

  4. Mukherjee S, Martin R, Sande B, Paige JS, Fajgenbaum DC. Epidemiology and treatment patterns of idiopathic multicentric Castleman disease in the era of IL-6-directed therapy. Blood Adv. 2022;6(2):359-367.

  5. van Rhee F, Voorhees P, Dispenzieri A, et al. International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease. Blood. 2018;132(20):2115 -2124.

  6. Referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for B-Cell Lymphomas V.3.2022. © National Comprehensive Cancer Network, Inc. 2022. All rights reserved. Accessed April 29, 2022. To view the most recent and complete version of the guideline, go online to NCCN.org. NCCN makes no warranties of any kind whatsoever regarding their content, use or application and disclaims any responsibility for their application or use in any way.

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Your report

A diagnosis of idiopathic multicentric Castleman disease (iMCD) requires the presence of multiple swollen or enlarged lymph nodes. In some patients, the swollen lymph nodes may not be visible and may require imaging tests to confirm their presence. These symptoms alone are not enough to diagnose iMCD, as they could be signs of other diseases.

Talk to your doctor if you have any questions about this report.

Additional information about iMCD is available for healthcare professionals at KnowiMCD.com/HCP.

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Download as PDF

Healthcare professionals can find information about the latest diagnostic criteria and treatment options by visiting

KnowiMCD.com/HCP

Your report

A diagnosis of idiopathic multicentric Castleman disease (iMCD) requires the presence of multiple swollen or enlarged lymph nodes. In some patients, the swollen lymph nodes may not be visible and may require imaging tests to confirm their presence. These symptoms alone are not enough to diagnose iMCD, as they could be signs of other diseases.

Talk to your doctor if you have any questions about this report.

Additional information about iMCD is available for healthcare professionals at KnowiMCD.com/HCP.

To protect your privacy, KnowiMCD.com does not save any of your information. Please be sure to download, save, and/or print this report.

Possible tests

In order to confirm a diagnosis of iMCD, a doctor will need to perform tests to detect the presence of multiple swollen lymph nodes.

  • Physical examination
  • Lab tests—blood or urine test
  • Imaging tests—CT scan, x-ray, MRI, PET scan
  • Excisional biopsy

By performing some of the above-mentioned tests, a doctor will be able to eliminate the possibility of autoimmune, cancerous, and infectious diseases. An excisional biopsy of the enlarged lymph node can confirm the diagnosis of iMCD.

The road to diagnosis

iMCD is a rare disease that can be difficult to diagnose because it can mimic so many other diseases. It may take a while to receive an accurate diagnosis because your doctor must rule out several different conditions before suspecting iMCD.

You may start your diagnosis journey at your primary care physician’s office, where you may be referred to a specialist.

Preparing for your appointment

Here are some tips to help you have a conversation with your doctor:

  • Write down any symptoms you’ve experienced, even if they may seem unrelated
  • Share the results of this Symptom Checker with your doctor

By performing some of the above-mentioned tests, a doctor will be able to eliminate the possibility of autoimmune, cancerous, and infectious diseases. An excisional biopsy of the enlarged lymph node can confirm the diagnosis of iMCD.

Start the conversation

Here are some tips to help you talk to your doctor about iMCD:

  • What kinds of tests might I need to confirm iMCD?
  • Do I need to plan for follow-up visits?
  • What treatments are available for iMCD? How will they affect my life?
  • Do you have any educational materials that I can take with me?
  • What else do I need to know about iMCD?

Notes:

Abbreviations: CT, computed tomography; MRI, magnetic resonance imaging; PET, positron emission tomography.

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Healthcare professionals can find information about the latest diagnostic criteria and treatment options by visiting

KnowiMCD.com/HCP

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Viral hemophagocytic lymphohistiocytosis.

A doctor who specializes in the branch of medicine that deals with rheumatic conditions.

Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes.

Positron emission tomography (PET) is a method of producing a 3-dimensional image of the internal structures of body tissues after administering a natural biochemical substance like glucose or fatty acids. These images are generated with the help of a computer.

A doctor who evaluates the changes caused by disease in tissues and body fluids and helps reach a diagnosis.

A doctor who specializes in the diagnosis and treatment of kidney abnormalities.

Macrophage activation syndrome.

Malignancy-associated hemophagocytic lymphohistiocytosis.

A disease in which cells of the lymph system grow excessively

Lymph nodes make immune cells that help the body fight infection.

A key signaling molecule that helps to regulate the body’s immune system.

Infectious disease medicine is the subspecialty of internal medicine that focuses on diagnosing and managing infections.

A rare condition that affects multiple groups of lymph nodes throughout the body. Patients with iMCD are HIV negative and HHV-8 negative.

The underlying cause or origin of a disease is unknown.

Hemophagocytic lymphohistiocytosis.

Human immunodeficiency virus.

Human herpesvirus-8.

A doctor who specializes in the branch of medical science that deals with cancers of the blood and blood-forming tissues.

Powerful molecules that are normally secreted by the cells of the immune system in response to infectious or noninfectious agents.

An overreaction of the body’s immune system in which too many cytokines are released into the bloodstream very quickly, with potentially severe or life-threatening effects.

Computed tomography (CT) is a method of producing an image of the internal structures by passing an x‑ray through the body.

The removal and examination of tissue, cells, or fluids from the body.

A condition in which the body’s immune system accidentally attacks itself.

Autoimmune lymphoproliferative syndrome.

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