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Splenic involvement in Niemann Pick disease

View larger version (157K): [in this window] [in a new window]   Figure 1. Patient is a 14-year-old female with Niemann Pick disease type C manifested primarily with seizures. This subcategory is a result of defective intracellular processing and transporting of low-density lipoprotein-derived cholesterol. She presented with acute abdominal pain and underwent abdominal exploration with splenectomy for splenic torsion. The spleen showed ischemic changes and was notable for a marked histiocytic proliferation, as illustrated in this image.

View larger version (131K): [in this window] [in a new window]   Figure 2. The image shows a marked histiocyte proliferation with these cells in clusters throughout the red pulp region of the spleen. Few residual lymphoid cells can still be identified. The histiocytes are abundant and have a foamy cytoplasm.

View larger version (131K): [in this window] [in a new window]   Figure 3. The ghost-like cells in the red pulp are consistent with the ischemic state and cell death secondary to the splenic torsion.

View larger version (133K): [in this window] [in a new window]   Figure 4. A higher-magnified view of a section of the previous image is shown. The cytoplasmic borders of the histiocytes are irregular and reflect ischemic changes secondary to the splenic torsion. A bland nucleolus can be seen in some of these cells.

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This Article Figures Only Services Similar articles in this journal Alert me to new content in the Image Bank Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Lazarchick, J. Search for Related Content PubMed Articles by Lazarchick, J. Related Collections Lipoidoses - Gaucher's, Nieman-Pick, etc. Related Image Bank Image Sets Social Bookmarking                   What's this?