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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 Bone marrow manifestations of inflammatory or other systemic (non-infectious disease) Lipoidoses - Gaucher's, Nieman-Pick, etc. Related Image Bank Image Sets Social Bookmarking                   What's this?

Bone marrow involvement in Niemann Pick disease

View larger version (131K): [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. The bone marrow aspirate and biopsy were obtained following splenectomy for splenic torsion. The marrow was hypercellular with marked hyperplasia of macrophages (histiocytes). The image shown illustrates the characteristic foamy cytoplasm of the histocytes in this disease.

View larger version (115K): [in this window] [in a new window]   Figure 2. Another lipid laden histocyte is shown. Myeloid and erythroid elements show normal maturation progression.

View larger version (119K): [in this window] [in a new window]   Figure 3. A high-power view of a typical histiocyte is shown.

View larger version (134K): [in this window] [in a new window]   Figure 4. The cytoplasmic features characteristic of this disorder are best illustrated in this view. Although clear and foamy in this case, the histiocytes may have a blue tint to the cytoplasm referred to as "sea blue histiocytes."

View larger version (168K): [in this window] [in a new window]   Figure 5. Low-power view of the marrow biopsy shows a cellular marrow with a marked increase in pale appearing histiocytes.

View larger version (149K): [in this window] [in a new window]   Figure 6. The same image is shown at higher magnification. Note that the nuclei of many of the histiocytes have a bland appearance and that the cytoplasm appears clear rather than foamy.

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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 Bone marrow manifestations of inflammatory or other systemic (non-infectious disease) Lipoidoses - Gaucher's, Nieman-Pick, etc. Related Image Bank Image Sets Social Bookmarking                   What's this?