Understanding iMCD and its impact
About iMCD
Castleman disease and its subtypes
Castleman disease (CD) is a rare condition that affects the immune system and is characterized by swollen lymph nodes. Bone marrow creates immune cells that help our bodies fight infections. In CD, there is an abnormal overgrowth of these cells, causing lymph nodes to swell and resemble a tumor. However, these growths are not cancerous.
There are 2 main types of Castleman disease:
Multicentric Castleman Disease (MCD)
MCD affects multiple groups of lymph nodes and can be treated with the help of a targeted therapy.
Unicentric Castleman Disease (UCD)
UCD involves a single group of lymph nodes and can be treated by surgically removing the swollen lymph node.
Idiopathic
multicentric Castleman disease (iMCD) is a subtype of MCD. Idiopathic means that the
cause or origin of the disease is unknown. People with iMCD are human immunodeficiency virus
(HIV) negative and human herpesvirus-8 (HHV-8) negative.
Every year, approximately 1000 to 1100 patients in the United States are diagnosed
with iMCD. It is rarer than amyotrophic lateral sclerosis (ALS) and cystic fibrosis (CF), and
iMCD can affect a person regardless of their age, gender, or race.
What happens in people with iMCD?
An abnormal growth in the lymph node—which can look like a tumor—is one of the reasons why iMCD can be mistaken for a cancerous condition like lymphoma. However, these growths are not cancerous.
The exact cause of iMCD is unknown. A protein called interleukin-6 (IL-6) may be elevated in patients, which is believed to be a common pathological driver of iMCD. IL-6 has been associated with the intensity of symptoms in patients with iMCD.
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.
It is important for patients to get an early diagnosis and receive treatment that can help minimize the damage caused by a cytokine storm.
Identifying the symptoms early can help get to the diagnosis faster.
Learn more about the major
symptoms of iMCD.
The road to diagnosis can be full of twists and turns
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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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