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Case 55 - Discussion

Hematopathology Case

 

  • The lymph node is markedly enlarged with follicular hyperplasia and interfollicular expansion. The follicles are variable in size and show frequent lymphocyte depletion with dendritic cell hyperplasia and intrafollicular vascular proliferation. The mantle zones are thickened with onion-skin pattern in some follicles. The interfollicular regions consist of mixed lymphocytes and prominent vascularity with hyalinization.

  • Diagnosis: Castleman disease, localized, hyaline vascular variant

 

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