Castleman disease Save

Understanding Castleman Disease

Castleman disease, also known as Castleman syndrome or angiofollicular lymph node hyperplasia, is a rare condition that affects the lymph nodes and related tissues. Named after the American pathologist Benjamin Castleman, who first described it in the 1950s, this disease has various subtypes, including unicentric Castleman disease (UCD) and multicentric Castleman disease (MCD).

In UCD, the disease is localized and affects a single lymph node region, while MCD involves multiple lymph nodes throughout the body. Although the exact cause of Castleman disease is unknown, it is believed to be related to an abnormal immune system response.

1. Types of Castleman Disease:
• Hyaline vascular Castleman disease: This is the most common subtype, usually affecting a single lymph node. It is often asymptomatic or causes mild symptoms.
• Plasma cell Castleman disease: This subtype is characterized by abnormal plasma cells that accumulate in the lymph nodes, leading to enlarged lymph nodes and potential organ dysfunction.
• Mixed Castleman disease: This subtype exhibits features of both hyaline vascular and plasma cell Castleman disease.

Castleman disease primarily affects adults, although it can also occur in children. It is more common in males than females, with most cases diagnosed between the ages of 30 and 40. The symptoms vary depending on whether it is unicentric or multicentric:

1. Symptoms of Unicentric Castleman Disease:
• Enlarged lymph nodes without symptoms (asymptomatic).
• Localized symptoms such as fatigue, night sweats, and weight loss may occur if the enlarged lymph node compresses nearby structures.
2. Symptoms of Multicentric Castleman Disease:
• Enlarged lymph nodes in multiple regions of the body, including the neck, groin, and armpits.
• Fever, night sweats, fatigue, and weight loss.
• Flu-like symptoms such as enlarged liver or spleen, anemia, and lower limb swelling.

Castleman disease is often diagnosed through a combination of physical examination, imaging tests, and lymph node biopsy. It is crucial to differentiate between the unicentric and multicentric forms, as the treatment approaches and prognosis differ significantly.

While the cause of Castleman disease remains unknown, ongoing research aims to better understand this condition and develop effective treatment options.