About Castleman Disease

Overview
Castleman disease is a rare condition characterized by the noncancerous enlargement of lymph node tissue. It can occur anywhere in the body and has different types, including unicentric and multicentric. Unicentric Castleman disease typically does not cause symptoms, while multicentric Castleman disease may cause various symptoms. Although Castleman disease is not considered cancer, individuals with this condition may sometimes develop non-Hodgkin lymphoma. The exact cause of Castleman disease is unknown, and optimal treatments for the multicentric type are still being studied. However, treatment for the unicentric type is highly effective. Castleman disease affects a relatively small number of people, with approximately 4,300 to 5,200 individuals in the United States receiving this diagnosis each year.
Causes and Risk Factors

The root causes of Castleman disease are not fully understood, but researchers believe that the different types of Castleman disease may have different underlying causes.

  • Unicentric Castleman disease (UCD) is thought to be caused by a genetic mutation called PDGFRB.
  • On the other hand, the exact cause of multicentric Castleman disease (MCD) is unknown, but it is often linked to an infection with human herpesvirus type 8 (HHV-8), which is more common in people with HIV or who are immunocompromised.

Non-modifiable risk factors for Castleman disease cannot be changed or controlled. These include:

  • Increasing age
  • HIV
  • Positive family history of Castleman disease or polyps

The modifiable risk factors of Castleman disease are not well-understood. However, certain lifestyle practices, such as smoking and exposure to environmental toxins, may potentially contribute to the development of this condition.

It's important to note that more research is needed to fully understand the modifiable risk factors of Castleman disease. It's always best to consult with your healthcare professional for specific information regarding your individual situation.

Symptoms

The most common early symptoms of Castleman disease include:

  • Unintentional weight loss
  • Fever
  • Fatigue
  • Swollen lymph nodes in the neck, armpit, collarbone, or groin
  • Pressure or a feeling of fullness in the stomach or chest
  • Anemia (low red blood cell count)

As Castleman disease progresses or reaches higher severity, other common symptoms that may occur include:

  • Night sweats
  • Vomiting
  • Loss of appetite
  • Stomach pain
  • Nerve damage
  • Enlarged liver or spleen
  • Pleural effusion (fluid buildup around the lungs)
  • Ascites (fluid buildup in the abdomen)
  • Pulmonary involvement (involvement of the lungs)
  • Systemic inflammation (inflammation throughout the body)

It's important to note that symptoms can vary depending on the type of Castleman disease and individual factors. If you experience any concerning symptoms, it's best to consult with a healthcare professional for an accurate diagnosis and appropriate management.

Diagnosis

To diagnose Castleman disease, the following examinations, tests, and procedures are commonly performed:

  • Medical history: Your healthcare provider will check your medical history before moving to any examinations, tests, and procedures.
  • Physical exam: Your healthcare provider will look for physical signs of the disease, such as pain, swelling, or the presence of enlarged lymph nodes or masses in your body.
  • Lab tests: Specific lab tests may be recommended, including blood draws to check for abnormalities that could suggest Castleman disease.
  • Imaging studies: Your healthcare provider may order imaging studies such as X-rays or MRI (magnetic resonance imaging) scans to get a better view of your body and identify any abnormalities or masses.
  • Clinical procedures: Based on the findings from the physical exam and initial tests, your healthcare provider may recommend additional procedures to help determine a diagnosis. The specific procedures will depend on the areas affected and may vary as such.

To determine the stage or severity of Castleman disease, additional examinations, tests, and procedures may be performed:

  • Further imaging studies: Additional imaging studies such as CT (computed tomography) scans or PET (positron emission tomography) scans may be ordered to evaluate the extent and location of disease involvement.
  • Biopsy: A biopsy involves taking a small sample of tissue from an affected area for further examination under a microscope. This can help determine the specific type and severity of Castleman disease.
  • Lymph node evaluation: Lymph nodes may be surgically removed and examined to help assess the stage and severity of the disease.

It's important to note that the specific examinations, tests, and procedures recommended for each individual may vary. It's best to consult with your healthcare provider for personalized guidance.

Treatment Options

The goals of treatment for Castleman disease (CD) are to control symptoms, prevent complications, and improve overall quality of life. The recommended treatment options depend on the type of CD and the specific circumstances of each individual. Here are some medication types, therapies, therapeutic procedures, and health behavior changes that may be recommended:

Medication types:

  • Immunotherapy: This involves the use of special antibody medications to target and shrink the abnormal immune cells in CD.
  • Chemotherapy: It is used to slow down the overgrowth of cancerous cells in the lymphatic system.
  • Corticosteroids: These can help reduce swelling and pain associated with CD.
  • Antiviral therapy: Antiviral medications can help control infections such as HHV-8 and HIV, which may be associated with CD.

Therapies:

  • Radiation treatment: It can be used to shrink tumor growth or destroy any remaining growth after surgery.
  • Surgical removal of affected lymph nodes: This is usually done in unicentric CD to prevent or treat life-threatening complications.

Health behavior changes:

  • Following a healthy lifestyle: Eating a balanced diet, exercising regularly, and getting enough rest can support overall health and well-being during CD treatment.

It's important to note that the specific treatment plan will vary for each individual based on their unique situation. It's recommended to consult with a healthcare professional who can provide personalized advice and guidance.

Medication dosing may be affected by many factors. Check with your health care professional about dosing for your individual situation. Other side effects can occur. Check with your health care professional or read the information provided with your medication for additional side effect information.