What is Castleman's Disease?

In 1956, a pathologist named Benjamin Castleman described a non-cancerous and extremely rare illness that could have a serious effect on the lymph nodes. This disease was later named after the doctor, though it has also been called angiofollicular hyperplasia or giant lymph node hyperplasia. Further studies in this area identified much more about Castleman or Castleman's disease, including that there are two main types known as unicentric and multicentric, and that there appear to be some risk factors for development of the illness. These risk factors are not always present in those who have either type of this condition.

As mentioned, Castleman's disease is a disorder of the lymph nodes, and in unicentric forms of the illness, a single lymph node is affected. Lymph cells in that node can overproduce, but this may or may not be noticed. Unicentric Castleman disease won’t necessarily cause any symptoms, and might evade diagnosis unless a person has physical exams or scans that discover the presence of an enlarged node. Multicentric Castleman's disease tends to have more symptoms and is less likely to be missed because many lymph nodes can have excess growth.

If unicentric forms of the disease do show symptoms these could include higher body temperature, tiredness, perspiration, decline in body weight, and low iron levels. These same symptoms may accompany multicentric Castleman's disease, but the more extensive illness can have additional expressions. People have notably large lymph nodes in areas like the neck, underarms and groin, many have persistent stomach upset, and some people develop damage to the nerves that feed the extremities resulting in numb sensations.

People may be more likely to notice symptoms because they might have other illnesses. Those who have HIV/AIDs have a higher risk of developing Castleman's. Yet, there are those who don’t have this illness and get it. Another potential risk factor is infection with a form of herpes virus called HHV8. It’s valuable to understand that while these are potential causal factors, they may not always be present, and the disease can occur at any time to anyone. Still, the rareness of the illness makes it extremely unlikely most people will ever have it.

Symptoms that might suggest Castleman's disease, especially when they persist, certainly warrant a visit to the doctor. Doctors may run many tests to determine diagnosis, but the gold standard is to test tissue from affected lymph nodes. If these show evidence of the disease, treatment can begin.

For unicentric forms of the illness, treatment tends to be removal of the lymph node that features overgrown tissue. Multicentric forms of the disease are more challenging to treat and might be addressed in a number of ways that are often highly individualized to each patient. Some treatments that could be considered include giving antiviral medications, treating with chemotherapy to arrest growth of lymph tissue, and using corticosteroids like prednisone to reduce inflammation. Clearly some of these medications can be challenging for the body to take, and this may be complicated when HIV is present too. Doctors must find a balance between trying to treat the disease without creating an additional burden of side effects.