Castleman disease (CD) is a rare and uncommon lymphoproliferative disorder that involves abnormal growth or enlargement of lymph nodes (part of the immune system). It is also known as Castleman’s disease or Castleman’s syndrome.
There are two main types of Castleman disease:
1. Unicentric Castleman Disease (UCD)
- Unicentric refers to one single lymph node or a group of lymph nodes being affected.
- It is the most common form of the disease.
- Symptoms may include:
- Swelling of the affected lymph node (often in the neck, armpit, or groin).
- Sometimes, patients might experience fever, night sweats, fatigue, and unexplained weight loss.
- Generally, the prognosis is good, and the disease can often be treated by surgical removal of the affected lymph node(s).
2. Multicentric Castleman Disease (MCD)
- Multicentric means that multiple lymph nodes or other organs are affected.
- This form is more severe and can be life-threatening.
- It may involve not only the lymph nodes but also other organs, including the liver, spleen, bone marrow, and lungs.
- Symptoms include:
- Fever, fatigue, night sweats, and unexplained weight loss (similar to UCD).
- Organ enlargement (liver, spleen).
- Anemia (low red blood cell count).
- Low blood pressure and difficulty breathing in severe cases.
- MCD is often associated with other conditions, such as HIV or human herpesvirus 8 (HHV-8), and can lead to complications like systemic inflammation and immunosuppression.
Symptoms of Castleman Disease:
- Lymph node enlargement (either localized or widespread).
- Fatigue, fever, night sweats.
- Unexplained weight loss.
- Shortness of breath.
- Anemia, low blood pressure.
- Abdominal pain (in cases with organ involvement like spleen or liver).
Diagnosis:
Castleman disease can be diagnosed through a combination of methods, such as:
- Physical examination: Lymph node enlargement or other signs.
- Blood tests: To check for signs of inflammation, anemia, and other abnormal blood counts.
- Imaging tests: CT scans or MRI to assess the size of lymph nodes or organs involved.
- Biopsy: A sample of the affected lymph node tissue is taken for microscopic examination to confirm the diagnosis and rule out other conditions.
Treatment:
The treatment approach depends on the type (unicentric vs. multicentric) and severity of the disease:
- Unicentric Castleman Disease (UCD):
- Surgical removal of the affected lymph node is often sufficient and can result in a cure.
- Multicentric Castleman Disease (MCD):
- Immunotherapy: Drugs like siltuximab (which targets interleukin-6, a protein involved in inflammation) may be used to reduce symptoms.
- Steroids: These may help manage inflammation and symptoms.
- Chemotherapy or antiviral treatments (especially if associated with HIV or HHV-8).
- Other immune-modulating drugs (such as rituximab) may be used in some cases.
Prognosis:
- Unicentric Castleman Disease (UCD) has a good prognosis with appropriate treatment (usually surgical removal).
- Multicentric Castleman Disease (MCD) can be more difficult to treat and may have a poorer prognosis, particularly if it is associated with underlying conditions like HIV or HHV-8 infection.
- The disease may lead to severe complications, but with appropriate management, the outcome can be improved.
