What is the definition of Castleman Disease?

Castleman disease is a rare condition that is characterized by an abnormal growth of multiple cells in a single lymph node or multiple lymph nodes (depending on the type of disease), causing them to enlarge. There are two main types of Castleman disease: 1) Unicentric Castleman disease (UCD) affects a single lymph node, usually in the chest or abdomen and is the most common form of the disease. 2) Multicentric Castleman disease (MCD) affects multiple lymph nodes and has two subtypes which are commonly associated with having been infected with the human immunodeficiency virus (HIV): 2a) Human herpes virus type 8 (HHV-8-associated MCD) 2b) Human herpes virus type-8 (HHV-negative or idiopathic MCD ), which is also known as Kaposi sarcoma-associated herpesvirus.

What are the alternative names for Castleman Disease?

There are several alternative names for Castleman disease, including angiofollicular lymph node hyperplasia, giant lymph node hyperplasia, and lymphoid hamartoma. Castleman disease may also be abbreviated to CD.

What are the causes for Castleman Disease?

While the exact cause is unknown, unicentric Castleman disease is currently believed to be caused by genetic mutations and inflammation. Multicentric Castleman disease can be caused by having been infected with human herpesvirus 8 (HHV-8) and human immunodeficiency virus (HIV). However, when the human herpesvirus 8 is not present, then the cause in unknown, in which case the disease is referred to as being idiopathic (of unknown cause) multicentric Castleman disease.

Advocacy Organizations

Castleman Disease Collaborative Network (CDCN) logo

The Castleman Disease Collaborative Network (CDCN) is a global initiative dedicated to accelerating research and treatment for Castleman disease and revolutionizing biomedical research.

What are the symptoms for Castleman Disease?

Patients with unicentric Castleman disease may not experience any symptoms, and the disease may only be detected when a single enlarged lymph node is discovered in the neck, chest, or abdomen during an examination. Some patients with unicentric Castleman disease may have symptoms similar to those in multicentric Castleman disease, such as fatigue, unexplained weight loss, nausea, skin rash, fever, night sweats, anemia, an enlarged liver and spleen (hepatosplenomegaly), and high levels of immune factors in the blood (hypergammaglobulinemia). Symptoms of multicentric Castleman disease include multiple areas of enlarged lymph nodes, unexplained weight loss, fatigue, loss of appetite, shortness of breath, nausea and vomiting, skin rash, fever, night sweats, anemia, an enlarged liver and spleen (hepatosplenomegaly), peripheral neuropathy (pain and tingling in arms, hands, legs, and feet), and high levels of immune factors in the blood (hypergammaglobulinemia).

What are the current treatments for Castleman Disease?

Treatment for Castleman disease depends on the type and severity. Treatment for unicentric Castleman disease involves surgery to remove the affected lymph node, which can cure the disease. If the lymph node is in an area that cannot be operated on (unresectable), then medication and ionizing radiation therapy (radiotherapy) may be used to destroy the affected lymph node. Treatment for multicentric Castleman disease depends on the severity and type and whether the patient is infected with the human immunodeficiency virus (HIV), human herpesvirus 8 (HHV-8), or both. A treatment plan may include medications to stop the cell overgrowth (immunotherapy; siltuximab , rituximab , IL-6 , sirolimus, or anakinra); chemotherapy; corticosteroids, such as prednisone, to control inflammation; antiviral drugs to block human immunodeficiency virus (HIV) or human herpesvirus 8 (HHV-8) or both; and bone marrow transplant (for severe disease).

What are the support groups for Castleman Disease?

There are several online, local, national, and international support groups for Castleman disease, including the following: Castleman’s Connect - https://www.castlemansconnect.com/connect-with-castleman-community/ Castleman Disease Collaborative Network - https://cdcn.org/patients-loved-ones/find-support/ Rare Connect: Castleman’s Disease - https://www.rareconnect.org/en/community/castleman-disease/members

What is the outlook (prognosis) for Castleman Disease?

The outcomes (prognosis) for unicentric Castleman are good as the disease can generally be cured with surgical removal of the affected lymph node. The outcomes (prognosis) for multicentric Castleman disease are varied as, in some patients, the disease can be characterized by periods of stable disease (not progressing) that can last for months or years. Other patients with multicentric Castleman disease may experience episodes of severe symptoms that may improve without treatment or respond to treatment, only to recur. Patients who are positive for the human immunodeficiency virus (HIV-positive) generally have poor outcomes (prognosis), as they have an increased risk of developing a severe form of multicentric Castleman disease that can progress rapidly, causing death within a period of weeks.

What are the possible complications for Castleman Disease?

Patients with unicentric Castleman usually do not experience any complications and can be cured after surgical removal of the affected lymph node. Complications for patients with multicentric Castleman disease may include life-threatening infections or organ failure. Patients with multicentric Castleman disease and human immunodeficiency virus (HIV)/ acquired immunodeficiency syndrome (AIDS) may experience severe, rapidly progressing disease and death. In rare instances, an affected lymph node in a patient with multicentric Castleman disease may form close to a blood vessel, causing an obstruction or aneurysm (ballooning of a blood vessel) requiring surgery. Having either type of Castleman disease can increase the risk of developing lymphoma (cancer of the lymph nodes).

When should I contact a medical professional for Castleman Disease?

If you discover an enlarged lymph node on the side of the neck, collarbone, under the arm, or in the groin area, or if you experience fatigue, unexplained weight loss, fever, or a persistent feeling of fullness in the chest or abdomen, make an appointment with your doctor as soon as possible.

How do I prevent Castleman Disease?

Since patients with human immunodeficiency virus are at an increased risk of developing human herpesvirus 8 (HHV-8) multicentric Castleman disease (HHV-8-associated MCD), avoiding infection with human immunodeficiency virus by practicing safe sex, limiting sex partners, avoiding injected street drug use and the use of dirty needles, and taking the pre-exposure prophylaxis pill (PrEP) can help to prevent HIV infection and reduce the risk of developing multicentric Castleman disease.
  • Condition: Kaposi Sarcoma (KS)-Associated Herpesvirus (KSHV)-Associated Multicentric Castleman Disease (MCD)
  • Journal: Blood advances
  • Treatment Used: High-Dose Zidovudine with Valganciclovir (AZT/VGC), Rituximab, or Rituximab with Liposomal Doxorubicin (R-Dox)
  • Number of Patients: 62
  • Published —
This study evaluated treatments for Kaposi sarcoma (KS)-associated herpesvirus (KSHV)-associated multicentric Castleman disease (MCD) in people living with HIV (PLWH).
  • Condition: Idiopathic Multicentric Castleman Disease (iMCD)
  • Journal: Journal of medical case reports
  • Treatment Used: Interleukin-6 Blockade
  • Number of Patients: 2
  • Published —
This case report discusses patients diagnosed with idiopathic multicentric Castleman disease (lymph gland inflammation; iMCD) treated with interleukin-6 blockade.