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Table 1 Comparison of Castleman disease subtypes

From: Multicentric Castleman disease of hyaline vascular variant presenting with unusual systemic manifestations: a case report

  Hyaline-vascular CD Plasma cell CD
Presentation Unicentric more common than multicentric Multicentric more common than unicentric
Distribution of lymphadenopathy Central (mediastinal, cervical) most common Central and peripheral lymph nodes
Age Third and fourth decades of life Unicentric form in young adults
Multicentric form in elderly (median sixth decade of life)
Prevalence Common Less common
Etiology and pathology Unknown; possibly follicular dendritic cell abnormalities IL-6, possibly plasma cell abnormalities, infection with HHV-8
Symptoms Incidental or occasional constitutional symptoms Frequent constitutional symptoms and hematological abnormalities
Clinical course Benign Usually aggressive
Organomegaly Rare Frequent (hepatosplenomegaly)
Associated lesions Paraneoplastic pemphigus, thrombotic thrombocytopenic purpura, plasmacytoid dendritic cell collections POEMS syndrome, HIV infection,
Kaposi sarcoma, amyloidosis, renal insufficiency
Progression to lymphoma Rare Common
Treatment Surgical resection, radiotherapy if inoperable Chemotherapy, antiviral therapy
  1. Abbreviations: CD Castleman disease, HHV Human herpesvirus, IL Interleukin, POEMS Polyneuropathy, organomegaly, endocrinopathy, multiple myeloma, and skin changes, HIV Human immunodeficiency virus