The Rarest of The Rare, Do You Know Castleman?

The lymphatic system is the body’s first line of defense against disease, so when lymphatic cells suddenly begin to multiply and overwhelm the immune system, it might be diagnosed as Castleman disease. Although scientists aren’t completely sure, there is evidence linking Castleman disease to a certain virus, called human herpesvirus 8 (HHV-8).

Only one risk has been confirmed by the medical community: If you have the HIV virus, you are more likely to get Castleman disease.

There are two classifications of Castleman disease. The unicentric, or localized, version usually develops around the patient’s 35th year, and patients often don’t have symptoms. This classification is typically diagnosed while the patient is being treated for a non-related illness. Surgery can help address the issues associated with this classification.

For the second classification, or multicentric, treatment isn’t clear-cut—more likely because there is an underlying comorbidity. Symptoms may include fatigue, enlarged lymph nodes, and an enlarged liver or spleen. Some patients report other symptoms such as vomiting, weight loss, and fever.

Testing for Castleman disease includes blood and urine tests, imaging tests, and a biopsy of the lymph node or nodes. The truth, though? Most of the tests are done to rule out other conditions such as non-Hodgkin lymphoma, Hodgkin lymphoma, and mesothelioma, for the simple reason that Castleman disease is so rare.


Do you know someone affected by Castleman disease? Share your insight with the Patient Worthy community by leaving a comment!

Erica Zahn

Erica Zahn

Erica Zahn is passionate about raising awareness of rare diseases and disorders and helping people connect with the resources that may ease their journey. Erica has been a caregiver, and is a patient, herself, so she completely relates to the rare disease community--on a deeply personal level.

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