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Gaucher's Disease

View larger version (154K): [in this window] [in a new window]   Figure 1. Marrow aspirate of a 15 month old child with a history of hypertonicity, aspiration pneumonia and hepatosplenomegaly. Two macrophages are shown which have a fibrillar, crumpled appearing cytoplasm and eccentric nuclei, consistent with Gaucher cells. The other hematopoietic elements present show normal maturation.

View larger version (119K): [in this window] [in a new window]   Figure 2. A higher power view of the same cells better illustrates the benign-appearing nuclei and the "crumpled tissue paper" appearance of the cytoplasm.

View larger version (127K): [in this window] [in a new window]   Figure 3. Although the cytoplasm is granular in the macrophages shown in this view, the characteristic striped appearance of Gaucher cells are less apparent. This variability is common in this disorder.

View larger version (166K): [in this window] [in a new window]   Figure 4. The striking proliferation of benign-appearing macrophages can readily be seen in this image. Interspersed normal hematopoietic elements are easily identified.

View larger version (174K): [in this window] [in a new window]   Figure 5. A higher power view of the Gaucher cells is shown.

View larger version (120K): [in this window] [in a new window]   Figure 6. Characteristically the cytoplasm of Gaucher cells show PAS postive staining which is diastase resistant. This is shown in the macrophage in the center of this image.

View larger version (155K): [in this window] [in a new window]   Figure 7. The cytoplasm of Gaucher cells also stain positive for acid phosphatase activity, which is tartrate resistant. A single macrophage is shown which is acid phosphatase positive. The beta glucocerebrosidase activity level was markedly decreased in this patient, confirming the diagnosis of Gaucher's disease.

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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 Lipid Storage Disorders: Gaucher's disease, Nieman Pick Related ASH-SAP Chapter Related Image Bank Image Sets Social Bookmarking                   What's this?