Castleman Disease Clinical Presentation

Updated: Jul 13, 2017
  • Author: Neetu Radhakrishnan, MD; Chief Editor: Emmanuel C Besa, MD  more...
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History and Physical Examination

Castleman disease can cause a variety of signs and symptoms or can remain asymptomatic. Unicentric Castleman disease and multicentric Castleman disease (MCD) tend to present differently.

Unicentric or localized Castleman disease is generally asymptomatic but may present as follows:

  • Localized lymphadenopathy with resultant compressive symptoms
  • Systemic manifestations like those of MCD

Signs and symptoms of MCD may include multiple lymphadenopathy and/or the following systemic manifestations, although some cases (<10%) are asymptomatic:

  • Fever
  • Night sweats (that soak the sheets)
  • Weight loss
  • Loss of appetite
  • Weakness
  • Fatigue
  • Shortness of breath, cough
  • Nausea and vomiting
  • Numbness and weakness (neuropathy)
  • Leg edema
  • Skin rashes
  • Hemangiomata
  • Pemphigus
  • Kaposi sarcoma

Other conditions associated with MCD include the following:

  • Amyloidosis
  • POEMS (polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell proliferative disorder, and skin changes) syndrome
  • Autoimmune disease
  • Hemolytic anemia
  • Immune thrombocytopenic purpura (ITP)
  • Acquired factor VIII deficiency

Some of these signs and symptoms symptoms might remit and relapse over time.

Amyloidosis, a condition in which abnormal proteins build up in tissues around the body, can occur in Castleman disease. This can lead to kidney damage; heart damage; nerve damage; and intestinal problems, mainly diarrhea. If the Castleman disease is treated successfully, the amyloidosis may improve or disappear.

Anemia can lead to problems such as fatigue and shortness of breath.

“B” symptoms, including severe fatigue, night sweats, fever, weight loss, and anorexia are typically present. These symptoms are typically driven by overproduction of interleukin-6 (IL-6). Overproduction of IL-6 also results in an acute-phase reaction, with elevation of the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level, elevated fibrinogen, thrombocytosis, and hypogammaglobinemia on laboratory testing..