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Case 86 - Discussion

Hematopathology Case

 

Castleman Disease

  • Localized Hyaline Vascular Type

    • AKA “angiofollicular lymphoid hyperplasia” or “giant lymph node hyperplasia”

    • Typically localized, mostly mediastinal lymph node, less commonly cervical , abdominal and axillary lymph nodes; average 6.0cm

    • Young adults, M=F, HIV-, clinically usually asymptomatic

    • Histology

      • Common perinodal fibrosis, sclerosis and fibrous bands

      • Thickened capsule and trabeculae, diminished or absent sinuses

      • Numerous small regressive germinal centers: predominantly FDCs and endothelial cells, often > 1 germinal center in one mantle, “lollipop”- penetrating blood vessels

      • Expanded mantle zones: “onionskin”- concentric rings

      • Prominent interfollicular vascularity, often hyalinized

      • Clusters of plasmacytoid dendritic cells

    • Immunostaining:

      • CD21/CD23/CD35 demonstrate expanded, disrupted, or concentrically arranged networks of follicular dendritic cells

      • Mantle cells may be CD5+

      • Polytypic plasma cells and follicles

    • Associated with FDC sarcoma

    • Localized diseases, treated with surgical resection and almost always curative

    • DDX: HIV-associated lymphadenopathy, angioimmunoblastic T-cell lymphoma, follicular or mantle cell lymphoma, and reactive lymphadenopathy

  • Localized Plasma Cell Variant

    • Wide age distribution, with median in young adults, M=F

    • Patients usually present with malaise, fevers, night sweats

    • Labs: cytopenia (anemia, thrombocytopenia), IL-6 increase, polyclonal hypergammaglobulinemia,

    • BM often has plasmacytosis

    • Most often found in abdominal lymph nodes

    • Association with amyloidosis and renal insufficiency

    • Morphologic features:

      • Follicles may be hyperplastic, normal, or atrophic

      • Marked interfollicular plasmacytosis, often with plasmacytoid immunoblasts

      • Interfollicular areas may be focally hypervascular

    • Surgical excision may be curative, but systemic symptoms may persist

  • Multicentric Plasma Cell Variant

    • Old age, median 50s, younger when associated with HIV

    • In multiple lymph node groups, splenomegaly +/- hepatomegaly

    • Presentations: malaise, fevers, night sweats

    • Labs: pancytopenia, hypoalbuminemia, IL-6 increase, hypergammaglobulinemia,

    • 15% have POEMS (Crow-Fukase disease): polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes

    • Often associated with HIV and HHV8

      • All HIV+ cases have HHV8

      • 40% of HIV- cases have HHV8

    • Patients usually have a poor prognosis