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Castleman's syndrome

Authoring team

Castleman's syndrome (Castleman Disease (CD)) is characterised by benign hyperplasia of lymph nodes:

  • is an uncommon benign lymphoproliferative disorder characterized by hyper-vascular lymphoid hyperplasia (1)
    • has no sex predilection, and the age of presentation ranges from 5 years to 70 years
    • ooccurs in any area of the body where lymph nodes are found, such as mesentery, axilla, groin, lung, pelvis and the retroperitoneum
    • been reported in the tongue, palate, lymph nodes within parotid gland parenchyma and capsule
    • mediastinum is the most common site of occurrence followed by head-neck region
    • isolated Castleman's disease in the neck is rare
  • can be subclassified as (2):
    • unicentric CD (UCD) involves one enlarged lymph node region
    • multicentric CD (MCD) involves multiple enlarged lymph node regions
      • can be associated with excessive cytokine production due to a plasma cell neoplasm (MCD–polyneuropathy, organomegaly, endocrinopathy, monoclonal paraprotein, skin changes) or uncontrolled human herpesvirus-8 infection (HHV-8) (HHV-8–positive MCD)
      • more than half of cases are idiopathic (1)
        • idiopathic MCD (iMCD) can be classified into three subtypes:
          • iMCD–thrombocytopenia, anasarca, fever, renal dysfunction/reticulin fibrosis, organomegaly (TAFRO)
            • hallmarks of this form are rapid onset cytokine storm with severe inflammation, anasarca, thrombocytopenia, and small volume lymphadenopathy, similar to hemophagocytic lymphohistiocytosis or sepsis
          • iMCD–idiopathic plasmacytic lymphadenopathy (IPL)
            • features include subacute or chronic lymphadenopathy, anemia of inflammation, and polyclonal hypergammaglobulinemia, often with increased IgG4 in serum and lymph node tissue;
          • iMCD-not otherwise specified (NOS)
  • both UCD and MCD may exhibit a wide range of symptoms that overlap with other immune-mediated conditions

POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, Skin changes) syndrome is a rare paraneoplastic disorder that may be associated with CD (1).

Management of CD

  • preferred management of unicentric CD is complete surgical excision, which is curative in approximately 95% of patients (1)
  • medical treatments for CD can be considered in three categories (2):
    • immunomodulators such as glucocorticoids, cytokine inhibitors, and sirolimus
    • antilymphoma therapies such as rituximab, cytotoxic chemotherapy, and BTK inhibitors
    • antimyeloma therapies such as thalidomide and bortezomib. The first-line therapy for all subtypes of iMCD is siltuximab, an IL-6 antagonist

Reference:

  1. Srivastava H, Reddy DS, Shah SN, Shah V. Castleman's disease. J Oral Maxillofac Pathol. 2020 Sep-Dec;24(3):593.
  2. Chen, L.Y.C., Zhang, L. and Fajgenbaum, D.C. (2025), Expert Perspective: Diagnosis and Treatment of Castleman Disease. Arthritis Rheumatol.

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The content herein is provided for informational purposes and does not replace the need to apply professional clinical judgement when diagnosing or treating any medical condition. A licensed medical practitioner should be consulted for diagnosis and treatment of any and all medical conditions.

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