Toolkit Modules:

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

Signs and symptoms of CD are determined by several factors including:

  • Disease extent (unicentric vs multicentric)
  • Pathology type (hyaline vascular vs plasma cell)
  • HIV status
  • Extent of IL-6 secretion
  • Associated immune phenomena
  • Overlap with POEMS syndrome 

With unicentric CD, clinicians can typically expect to observe: 

  • 1 lymph node station affected
  • A mediastinal mass (alternate sites include intra-abdominal masses or involvement of cervical, axillary, and inguinal nodes).
  • Asymptomatic patients, unless a compression on neurovascular site or other organs cause symptoms
  • Patients who are mostly HIV negative with a hyaline vascular pathology and a lack of systemic symptoms or laboratory abnormalities reflecting excess IL‐6 Secretion 

With multicentric CD, clinicians can typically expect to observe:

  • Multiple lymph node stations affected
  • Generalized lymphadenopathy
  • 80% frequency of liver enlargement and 65% frequency of spleen enlargement
  • PC‐variant histology
  • Symptoms including fever, night sweats, fatigue, anorexia, and weight loss 

Disorders associated with MCD include:

  • Decreased albumin production
  • Edema
  • Pleural effusion
  • Ascites
  • Venous thrombosis
  • Hemangiomata
  • Skin lesions
  • POEMS (NOTE: POEMS is associated with endocrine abnormalities, monoclonal gammopathy, sclerotic bone lesions and, in some cases, sensory, glove and stocking neuropathy)

Increased frequency of clonal hematologic disorders could include:

  • Myeloma
  • Amyloidosis 
  • Laboratory findings for MCD include:
  • Anemia or thrombocytopenia
  • Elevated ESR
  • Increased C‐reactive protein
  • Increased IL-6
  • Increased fibrinogen
  • Proteinuria
  • Abnormal thyroid function tests
  • Hypergammaglobulinemia
  • Thrombocytosis
  • Frequently, elevated plasma VEGF
  • Low titer of antinuclear antibodies in about 30% of patients