PMC3420507_01
Morphologic features of lymph node. Images are from the antemortem lymph node biopsy. (a and b) CD20 and CD68 immunoperoxidase stains highlight the B-cell-rich and histiocyte-rich regions, the lymph node corresponding to areas involved by marginal zone B-cell lymphoma (a) and Erdheim-Chester disease (b), respectively, 100x magnification. (c and e) lymphoplasmacytic infiltrate present in the B-cell-rich areas, hematoxylin, and eosin, 200x and 400x magnification. (d and f) Histiocytic infiltrate with occasional Touton type giant cells in histiocyte-rich-regions, 200x and 1000x magnification. (g and h) In situ hybridization for kappa (g), and lambda (h), in lymphoplasmacytic areas of lymph node demonstrating lambda predominance, 100x magnification.
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fig1
 Shortly thereafter, a cervical lymph node biopsy was performed that revealed an effaced lymph node with a dimorphic morphologic picture, Figure 1. A portion of the node demonstrated a diffuse proliferation of small lymphocytes with plasmacytoid features (Figures 1(a), 1(c), 1(e), 1(g), and 1(h)). Other regions of the lymph node demonstrated a diffuse proliferation of histiocytic cells with pale staining cytoplasm and bland nuclei (Figures 1(b), 1(d), and 1(f)). Only very rare giant cells were observed (Figure 1 (d)).
Gross and microscopic appearance of heart. Cross-section of heart, left, demonstrating extensive tan-yellow marbling of the myocardium due to infiltration by histiocytes. Microscopic appearance of heart, right. There was dense proliferation of histiocytes within the myocardium similar to that seen in the lymph node. Hematoxylin and eosin, 100x magnification.
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fig2
 Autopsy findings demonstrated extensive myocardial and peripancreatic adipose tissue infiltration by histiocytic cells similar to that observed in the antemortem lymph node biopsy, Figure 2.