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FAQs and Glossary

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Frequently Asked Questions (FAQs)

  • What does idiopathic mean?

    The term idiopathic means that the underlying cause or origin of a disease is unknown.

  • What is Castleman disease?

    Castleman disease (CD) is a rare condition in which cells of the lymph nodes grow rapidly but are not cancerous. If not diagnosed and treated immediately, it can lead to organ failure or other harmful conditions.

  • What are the 2 main types of Castleman disease?

    Unicentric Castleman disease (UCD): Affects only a single lymph node. Multicentric Castleman disease (MCD): Affects multiple lymph nodes throughout the body. Many patients experience this in their neck, armpit, stomach, or groin areas.

  • What is iMCD?

    Idiopathic multicentric Castleman disease (iMCD) is a serious, complex, and treatable condition. It affects multiple groups of swollen lymph nodes, causing them to swell, and the symptoms can mimic cancerous, autoimmune, infectious, and various other disorders.

  • What happens in people with iMCD?

    It is thought that symptoms experienced by people with iMCD may be caused by a phenomenon called a cytokine storm. A cytokine storm is an event in which multiple cytokines are released too quickly in the body. Normally, immune cells produce these proteins to help our bodies fight infections.

    In people with iMCD, a particular cytokine called interleukin-6 (IL-6) may be responsible for causing flu-like symptoms, fever, night sweats, fatigue, and organ failure.

  • What are the common symptoms of iMCD?

    The common symptoms of iMCD include the following:                   

    • Swollen lymph nodes
    • Fatigue
    • Anemia
    • Enlarged organs
    • Night sweats
    • Fever
    • Unintended change in weight
    • Flu-like symptoms
    • Rash
    • Fluid accumulation
  • Is iMCD a form of cancer?

    iMCD is not cancer; however, it can mimic the symptoms of many other diseases, including some malignancies such as lymphoma, and can be challenging to diagnose.

  • Who can help diagnose iMCD?

    A patient usually consults a primary care physician (PCP), who may conduct various tests that can make them suspect cancerous, autoimmune, infectious, or various other diseases. Based on their findings, your PCP may refer you to either a rheumatologist, a hematologist-oncologist, or an infectious disease expert who can help you get to a diagnosis. A hematologist-oncologist is the one who may eventually diagnose iMCD with the help of a pathologist.

  • How is iMCD diagnosed?

    Physical examinations and various tests like blood work, x-rays, PET scans, CT scans, and biopsies will be performed in the diagnostic process. Eventually, an excisional biopsy of a swollen lymph node will be performed, which is the only definitive way to diagnose iMCD.

  • Are there any guidelines that can help diagnose iMCD?

    For years, little has been known about iMCD, which has made the diagnosis and treatment journey frustrating for patients. Now doctors have access to new data and guidelines that may help them in their diagnostic process.

  • Are there any treatments or medications for iMCD?

    Here are some treatments that doctors are currently using to help patients manage iMCD:

    • Monoclonal antibodies
    • Corticosteroids
    • Chemotherapy
    • Immunotherapies
    • Radiation
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Glossary

ALPS:

Autoimmune lymphoproliferative syndrome.
Autoimmune condition:

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

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

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

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.
Cytokines:

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

During this surgical procedure, an incision/cut is made in the skin to remove a swollen lymph node, which is then examined under a microscope to look for signs of a disease. A small amount of healthy tissue around the abnormal area may also be removed.
Fine needle aspiration (FNA) or core biopsy:

A procedure during which tissue is obtained from a swollen lymph node with the help of a fine needle and syringe.
Hematologist-oncologist:

A doctor who specializes in the branch of medical science that deals with cancers of the blood and blood-forming tissues.
HHV-8:

Human herpesvirus-8.
HIV:

Human immunodeficiency virus.
HLH:

Hemophagocytic lymphohistiocytosis.
Idiopathic:

The underlying cause or origin of a disease is unknown.
Idiopathic multicentric Castleman disease (iMCD):

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

Infectious disease medicine is the subspecialty of internal medicine that focuses on diagnosing and managing infections.
Interleukin-6 (IL-6):

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

Lymph nodes make immune cells that help the body fight infection.
M-HLH:

Malignancy-associated hemophagocytic lymphohistiocytosis.
MAS:

Macrophage activation syndrome.
Nephrologist:

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

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

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.
POEMS syndrome:

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

A doctor who specializes in the branch of medicine that deals with rheumatic conditions.
V-HLH:

Viral hemophagocytic lymphohistiocytosis.
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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.

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

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.

© 2022 EUSA Pharma (US) LLC. All rights reserved.
US-NPS-2200024      1/22

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.

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.

A procedure during which tissue is obtained from a swollen lymph node with the help of a fine needle and syringe.

During this surgical procedure, an incision/cut is made in the skin to remove a swollen lymph node, which is then examined under a microscope to look for signs of a disease. A small amount of healthy tissue around the abnormal area may also be removed.

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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