Skip to main content

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