Toolkit Modules:

1. Disease Overview
2. Pathology of Castleman Disease
3. Clinical Manifestations
4. Important Differential Diagnoses
5. Case Studies

Castleman disease (CD) is a lymphoproliferative disorder which displays a great variation in presenting symptoms and signs. In some cases, it may result in serious complications which can lead to death.

CD is clinically manifested as either Unicentric or Multicentric.

Unicentric CD, or UCD, is localized to one lymph node station, although occasionally small, regional, satellite nodes maybe present. It generally is seen in an adult population, with the median age in the fourth decade. It is typically a symptomatic, and is curable by surgery in 95% of patients.

Multicentric CD, or MCD, is widespread, aggressive, has high morbidity and is associated with an adult population in the fifth to sixth decade.

Variations in the lymph node tissues of patients with CD have led to 4 histological classifications:

  •  Hyaline vascular variant
  •  Plasma cell variant
  •  Mixed cell variant
  •  Plasmablastic variant

Most cases of UCD are of the hyaline vascular variant. The plasma cell variant is most often found in MCD.

MCD can further be categorized into:

  •  HHV-8-positive/HHV-8-associated
  •  Idiopathic

These 2 MCD categories are distinct and prognosis differs.

CD is a rare and incompletely understood disease. While most cases tend to be managed in a community setting, there has been an increased tendency to refer CD Patients to centers of excellence where physicians with specific CD experience can confirm diagnosis, advise on disease management, and outline a treatment plan. Correct and timely diagnosis of the disease may improve the prognosis of CD patients.