About Castleman Disease

Many patients and doctors have questions about Castleman disease. We are here to help share what is known and to push forward research to answer those questions that are not yet answered.
Overview

Castleman disease lymph node under the microscope

Castleman disease (CD) is a group of three immune system disorders that share a similar lymph node appearance under the microscope, but have different symptoms, causes, and treatments. The three main subtypes of CD include:

  1. Unicentric Castleman disease (UCD)
  2. HHV-8-associated multicentric Castleman disease (HHV-8+MCD)
  3. HHV-8-negative/idiopathic multicentric Castleman disease (iMCD)

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Incidence Rate

6,500-7,700

New cases per year

 

CD is a rare disease but it does not discriminate—it can occur in people of all ages and genders. In the USA, there are an estimated 6,500 to 7,700 new CD cases diagnosed per year. For comparison, there are about 220,000 new cases of lung cancer and 22 million diagnoses of diabetes every year. It is therefore easier to understand why most physicians may not have much, or any, experience in treating CD.

Causes

All cases of unicentric Castleman disease (UCD) and HHV-8-negative multicentric Castleman disease (iMCD) are “idiopathic,” which means that the cause is not known. There are also no known risk factors for UCD or iMCD, though the possibility of a genetic cause is being researched. In HHV-8-associated multicentric Castleman disease, the HHV-8 virus causes and drives the disease.

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Signs & Symptoms

Many of the symptoms of Castleman disease overlap with other more common illnesses. Castleman disease ultimately must be diagnosed with a lymph node biopsy and cannot be diagnosed by symptoms alone.

  • Lymph node enlargement
  • Flu-like symptoms such as fevers, night sweats, fatigue, and weight loss
  • Anemia (low red blood cells or low hemoglobin)
  • Elevated C-reactive protein (CRP) levels
  • Very low or very high platelets
  • Kidney Dysfunction
  • Liver Dysfunction
  • Fluid Accumulation
  • Hypergammaglobulinemia (elevated antibodies in the blood)

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Diagnosis & Treatment

Carol, Stacey, and Gary blood draw sample donationDiagnosis of Castleman disease requires a lymph node biopsy, and each subtype of Castleman disease requires a different treatment.

Treatments may include surgery and/or immunotherapies, which are medications that act on the immune system. Severe cases of Castleman disease may also require chemotherapy.

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Prognosis

Most patients with unicentric Castleman disease (UCD) do very well with treatment. The average  length of survival after UCD diagnosis is greater than 10 years, and life expectancy is usually not changed by UCD.

Patients with multicentric Castleman disease (MCD) have a 5-year overall survival rate of 65% (the study did not separate patients into HHV-8+MCD and iMCD), but more more research is needed to provide further information about overall prognosis.

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Frequently Asked Questions

Answering your questions about CD

Many patients and doctors have questions about Castleman disease. We are here to help share what is known and to push forward research to answer those questions that are not yet answered.

Frequently Asked Questions
Newly Diagnosed

Do you have a new diagnosis of CD?

A new diagnosis of Castleman disease can be a lot to take in and you likely have many questions about your diagnosis and what your Castleman disease journey may look like. We are here to help.

Newly Diagnosed
Patients & Loved Ones

Supporting people affected by CD

We strive to ensure that people living with CD are educated about their disease, feel connected to community, have access to experienced physicians, are informed of research underway and know how to join the fight! We hope that you will leverage our resources and engage with the CDCN to find the best possible care and to help us find a cure.

Patients & Loved Ones
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