Castleman disease


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  • Prognosis

    • unicentric (best)
    • idiopathic multicentric
    • HHV8+ multicentric (worst)

  • Unicentric

    • one or more nodes
    • morphology: hyaline vascular (80-90%) > plasma cell
    • younger age

  • Multicentric

    • multiple nodal regions
    • morphology: hypervascular to plasma cell

  • Hyaline vascular

    • hyaline: in regressed GCs
    • vascular: lollipop lesions
    • other: onion skinning mantle zones, multiple GCs per follicle
    • architecture: follicular hyperplasia, interfolllicular plasma cells, lymphs, immunoblasts, eos
    • younger, usually asx

  • Plasma cell variant

    • plasma cell: interfollicular plasmacytosis in sheets
    • other: vascular proliferation, hyperplastic GCs
    • architecture: plasma cell sheets, widely spaced reactive follicles, vascular proliferation (more pronounced in hhv8+)
    • HHV8 positivity is more aggressive, plasmablastic, risk prog to PBL
    • Clinical: B sx, immunodeficiency association
    • Pseudo monotypic lambda bcz HHV8 replicates in Lambda B cells
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