Understanding Castleman’s Disease

Castleman’s Disease (CD) is a rare lymphoproliferative disorder that was first recognized in the 1950s.1 Also referred to as angiofollicular or giant lymph node hyperplasia, CD exists in 2 distinct forms—Multicentric Castleman’s Disease (MCD), which affects multiple lymph nodes throughout the body, and Unicentric Castleman’s Disease (UCD), which affects one or more lymph nodes in a localized area, most commonly in the chest, abdomen, or axillary region.1,2 In addition to the numbers and location of affected lymph nodes, symptoms and histologic characteristics differ between the 2 types, as described in the table below.1,2

Characteristics of Multicentric and Unicentric Castleman’s Disease

Multicentric Castleman’s Disease

Unicentric Castleman’s Disease

Affects multiple lymph nodes throughout the body1

Histologic characteristics: concentric sheets of plasma cells surround germinal centers1

Associated with an array of systemic symptoms, including3,4:

  • Fever, night sweats
  • Anorexia/weight loss
  • Hepatomegaly/splenomegaly
  • Pericardial/pleural effusions

Affects a single group of lymph nodes, most commonly in chest, abdomen, or axillary regions1,2

Histologic characteristics: hyalinization and hypervascularity in germinal centers1

Localized symptoms associated with compression in affected area—systemic symptoms are uncommon3

CD is not considered a malignant disease; however, affected patients are at higher risk of developing lymphoma. Increased incidence of Hodgkin lymphoma is associated with both UCD and MCD, whereas a higher risk of non-Hodgkin lymphoma is more frequently associated with MCD.5

Although CD can affect patients at any age, most patients with the multicentric form of the disease are between 50 and 60 years of age at the time of diagnosis. The majority of patients with the unicentric form are diagnosed between 30 to 40 years of age.2,3

For additional information about Castleman’s Disease, including research highlights, visit the Publications and Events section of this website.

  1. Casper C. The aetiology and management of Castleman disease at 50 years: translating pathophysiology to patient care. Br J Haematol. 2005;129(1):3-17.
  2. Roca B. Castleman’s disease: a condition with protean manifestations. Onkologie. 2011;34(8-9):412-414.
  3. Dispenzieri A, Gertz MA. Treatment of Castleman’s disease. Curr Treat Options Oncol. 2005;6(3):255-266.
  4. El-Osta HE, Kurzrock R. Castleman’s disease: from basic mechanisms to molecular therapeutics. Oncologist. 2011;16(4):497-511.
  5. van Rhee F, Stone K, Szmania S, Barlogie B, Singh Z. Castleman disease in the 21st century: an update on diagnosis, assessment, and therapy. Clin Adv Hematol Oncol. 2010;8(7):486-498.