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 |