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Case 144 - Discussion

Uploaded: 2010-10-10, Updated: 2010-10-10

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  • Diagnosis: reactive lymphoid hyperplasia with Castleman-like changes

  • Discussion: This is a markedly enlarged lymph node with both hyperplastic and atrophic follicles with prominent expansion of interfollicular areas. The interfollicular areas contain a mixed population of cells with marked increased vascularity. There are some features of Castleman disease. The patient had no history of HIV, and the lymph node was negative for EBV and HHV8 stains. Flow cytometry didn't show any monoclonal lymphocytes. For any lymph node with Castleman-like change, several major differential diagnoses should be considered:

    1. Castleman disease: typically atrophic follicles with depletion of germinal center lymphocytes and hyperplasia of vessels and germinal center dendritic cells;

    2. Classic Hodgkin lymphoma, lymphocyte-rich subtype, with nodular growth pattern; the lymph node shows partially preserved architecture with nodular pattern. The nodules consist of B cells and often regressed, eccentrically placed germinal centers, reminiscent of Castleman's disease; scant or rare tumor cells typically present in the expanded mantle zones in a dispersed pattern. Mostly classic RS cells, LP cells may occur;

    3.  IgG4-related lymphadenopathy, type I, with multicentric Castleman disease-like features:

 
IgG4-related lymphadenopathy
Multicentric Castleman Disease
Presentation
Typically asymptomatic w/ or w/o IgG4 related disease. No constitutional symptoms
Abrupt or gradual onset of symptoms, with multiple lymphadenopathy and constitutional symptoms
Age
Middle-aged or elderly, median age 69 years
Middle-aged or elderly, median age 64 years
Gender
Marked male predilection, M:F=8:1
M:F=2:1
HIV
Negative
Often positive
Serum Ig
↑IgG, ↑ IgG4, ↑ IgE, normal IgM and IgA
↑IgG, ↑ IgM, ↑ IgA
Serum IL6
Normal or mildly increased
Increased
CRP
Normal or mildly increased
Increased
Anemia
No or mild
Common, often moderate to severe
Histology
Preserved architecture. Normal to hyperplastic follicles. Interfollicular zone often rich in plasma cells and eosinophils
Preserved architecture. Usually small and regressed lymphoid follicles. Interfollicular florid plasmacytosis, not rich in eosinophils
IHC
↑IgG4+ cells and IgG4/IgG ratio;
No light chain restriction
No or ↑IgG4+ cells, may have lambda light chain restriction, HHV8 often +
Prognosis
Benign clinical course; good response to steroid therapy
Unpredictable clinical course, but often with high mortality