Castleman Disease Services

Castleman disease describes a group of rare disorders that involve enlarged lymph nodes and a broad range of inflammatory symptoms and laboratory abnormalities. Castleman disease can involve one or more enlarged lymph nodes in a single region of the body (unicentric CD, UCD) or it can involve multiple enlarged lymph node regions (multicentric CD, MCD).

Castleman disease (CD) can be difficult to diagnose as it is both a rare disease and it can have similar signs and symptoms as some cancers, infectious diseases, and autoimmune diseases. There are different types of Castleman disease which are classified based on the underlying cause of CD.

1. Unicentric Castleman disease or UCD involves a single enlarged lymph node or multiple enlarged lymph nodes within a single region of the body that display microscopic features consistent with Castleman disease. It is also sometimes called localized Castleman disease.
2. Multicentric Castleman disease or MCD has multiple regions of enlarged lymph nodes. MCD involves characteristic microscopic features in the enlarged lymph nodes, flu-like symptoms, and organ dysfunction due to excessive cytokines or inflammatory proteins. MCD should be further classified into three categories based on the underlying cause: POEMS-associated MCD, HHV-8-associated MCD and HHV-8-negative or idiopathic MCD (iMCD).
   1. POEMS-associated MCD: a cancerous cell population found in patients with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes) can cause MCD in a fraction of patients.
   2. HHV-8-associated MCD: HHV-8 is a common virus that does not result in disease for the overwhelming majority of individuals that are infected with it. The fraction of individuals with HHV-8-associated MCD are HIV positive or have suppressed immune systems that are unable to control the HHV-8 virus.
   3. Idiopathic MCD (iMCD): iMCD, which is the most common form of MCD, occurs for an unknown cause. iMCD also has important differences in symptoms, disease course, and treatment from POEMS-associated MCD and HHV-8-associated MCD.

      iMCD can be further sub-classified intro three clinical subgroups:

      • iMCD with TAFRO syndrome (iMCD-TAFRO) is characterized by acute episodes of Thrombocytopenia, Anasarca, Fever, Renal dysfunction or myelofibrosis, and Organomegaly.
      • iMCD with idiopathic plasmacytic lymphadenopathy (iMCD-IPL) is characterized by thrombocytosis, hypergammaglobulinemia, and a more chronic disease course.
      • iMCD, not otherwise specified (iMCD-NOS) is diagnosed in iMCD patients without TAFRO syndrome or iMCD-IPL.

Facts about Castleman Disease

• It affects approximately 4,300-5,200 patients of all ages, genders, and ethnicities each year in the U.S.
• MCD has a five-year survival rate of 65-75 percent, but there is reason to believe that this will continue to improve with breakthrough therapies such as siltuximab.
• CD can range from enlargement of a single lymph node with flu-like symptoms to multiple enlarged lymph nodes with failure of every vital organ (liver, kidneys, bone marrow).

More information about Castleman disease