Castleman’s disease

Castleman’s disease

Castleman’s disease is a lympho-proliferative disorder characterized by an overgrowth of the cells belonging to the lymph system. This disorder may be unicentric (localized to a certain area) or multicentric (widespread involvement). Castleman’s disease is not a ‘cancer’ per se, but it behaves like 1 in many ways.

Causative & risk factors

The exact cause of Castleman’s disease is not known. But it has been found that many patients with this disorder are infected with herpes virus 8 (HHV8). HIV positive people have a higher risk of catching HHV8 infection and thus of developing Castleman’s disease.

 

Clinical presentation

In individuals with unilateral CD, a single lymph node is affected, which becomes enlarged. Depending upon the location, there may be symptoms arising in the neck, chest or abdomen caused as a result of pressure from the enlarged lymph node. Systemic symptoms may occur in some people causing fever, night sweats and fatigue.

Persons with multicentric CD present with several enlarged lymph nodes, especially in the neck, groin or axillae. The patient develops systemic symptoms such as fever with night sweats and excessive fatigue. The patient starts losing his appetite and weight loss occurs. The patient may develop hepatomegaly and/or splenomegaly. Some patients may develop skin rashes and peripheral neuropathy.

 

Investigations

Imaging scans such as CT or MRI of the affected areas are carried out. A PET scan may be done. A lymph node biopsy is performed to confirm the diagnosis of Castleman’s disease.

 

Treatment  

Localized forms of Castleman’s disease can be treated surgically. Surgery can be followed up with radiation therapy. The widespread form of CD is treated with a combination of medications and chemotherapy.

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