Understanding the different types of Castleman disease

Understanding the different types of Castleman disease
Castleman disease is named after Dr. Benjamin Castleman who first described the condition in the 1950s. Alternatively known as giant lymph node hyperplasia and angiofollicular lymph node hyperplasia, Castleman disease is not cancer. It is a group of three heterogeneous inflammatory disorders that produce excess molecules primarily affecting the lymph node system. The condition also exhibits flu-like symptoms, lymph node enlargement, and can result in vital organs becoming dysfunctional.

There are two primary types of Castleman disease with the number of lymph nodes affected taken as the basis for the classification. Further, each primary type has a subtype of the lymph node condition as the lymph tissue is found in many places throughout the body including the lymph nodes, thymus, spleen, tonsils & adenoids, bone marrow, and digestive tract among other locations.

Note that there are no known causes of Castleman’s disease. It is a rare disorder that prompts an overgrowth of the lymph nodes in various parts of the body that can lead to further complications. In most cases, the Human Herpesvirus 8 is identified as the main trigger of Castleman’s disease. However, there are significant risk factors that can aggravate the unnatural overgrowth of cells in the lymph nodes without warning. People in their early and mid-thirties are at risk of developing the unicentric Castleman disease and those above the age of fifty are at a higher risk of developing multicentric Castleman disease. This article focuses on explaining the disease in brief along with covering its types below:

Unicentric Castleman Disease
Localized or unicentric Castleman disease is one of the more common types that affect a single group of lymph nodes. The chest or abdomen lymph nodes are affected and the condition prompts unnatural growth of the lymph nodes. The enlarged growth can affect one’s breathing as the chest presses against the trachea (windpipe) and bronchi (smaller wind tubes). Enlarged nodes in the abdomen can cause pain and affect one’s appetite as there will be associated problems with eating. The lymph nodes in the neck, groin, and underarm area are also affected by visible indicators including the growth of lumps under the skin.

Multicentric Castleman Disease
A multicentric condition disturbs a group of lymph nodes and other organs that carry the lymphoid tissue. It mostly affects people who have been previously infected with the Human Immunodeficiency Virus (HIV). Infection, fever, weight loss, night sweats, and fatigue are among the more common symptoms of Multicentric Castleman disease. Severe cases can even cause nerve damage resulting in weakness and numbness. It is also one of the types that increases the risk of developing lymphoma, a cancer of the lymphoid tissue that is life-threatening with fewer treatment options.

Subtypes of Castleman diseases
It can also be classified on the basis of how the tissue looks under the microscope:

  • Hyaline vascular is a localized Castleman disease with fewer symptoms.
  • Plasma cell type is a multicentric variant that exhibits multiple symptoms indicating a developing condition.
  • Mixed microscopic subtype exhibits symptoms of both hyaline vascular and plasma cell subtypes.
  • Plasmablastic cell subtype has a less favorable outlook with fewer symptoms.
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