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Chediak-Higashi Syndrome

View larger version (91K): [in this window] [in a new window]   Figure 1. Peripheral smear from a 17 year old female with Chediak-Higashi syndrome is shown. Multiple large azurophilic granules characteristic of this disorder can be seen in the cytoplasm of the neutrophil.

View larger version (100K): [in this window] [in a new window]   Figure 2. Multiple large azurophilic granules are present in the cell shown. It is not clear whether this is an abnormal segmented neutrophil or a lymphocyte.

View larger version (162K): [in this window] [in a new window]   Figure 3. The marrow is markedly cellular as illustrated in this intermediate power view. There is cellular heterogeneity with erythroid progenitors at all stages of maturation, background increase in small lymphocytes and left-shifted myeloid series with an increased number of eosinophils.

View larger version (99K): [in this window] [in a new window]   Figure 4. A higher power view of the previous image shows the abnormally large granules in the eosinophil precursors and the neutrophils. In addition, giant granules are present in vacuoles in the cytoplasm of the myeloid precursors. Dysplastic changes can be seen in the nucleated erythroid elements. Finally, there is a background increase in normal appearing lymphocytes.

View larger version (74K): [in this window] [in a new window]   Figure 5. Similar abnormalities in the myeloid elements are prominent in this view. Variable numbers of giant azurophilic granules are noted in the cytoplasm of early myeloid precursors and abnormally eosinophilic granules can be seen in the eosinophils.

View larger version (82K): [in this window] [in a new window]   Figure 6. All the neutrophils in this view show the characteristic primary granule abnormality of Chediak-Higashi syndrome. The two myeloid precursors show various stages of abnormal granule formation in this disorder.

View larger version (87K): [in this window] [in a new window]   Figure 7. A composite of the granule abnormality can be seen in this view. Neutrophils, bands and metamyelocytes have abnormal granules. Two cells illustrate the granule abnormality typical of eosinophils and eosinophilic precursors in this disorder. The myeloid precursor noted in the center of the image also has multiple large azurophilic granules in its cytoplasm.

View larger version (84K): [in this window] [in a new window]   Figure 8. A similar high power view of another area of the aspirate best illustrates the primary granule abnormalities of the precursor and terminally differentiated myeloid lineage that are the hallmark of this disorder.

View larger version (122K): [in this window] [in a new window]   Figure 9. The bone marrow touch prep is shown. Numerous vacuolated myeloid precursor cells are evident. Many of the vacuoles contain large azurophilic granules. Similar cytoplasmic granules can also be seen in the neutrophil band forms.

View larger version (157K): [in this window] [in a new window]   Figure 10. A low power view of the biopsy shows it to be approximately 90% cellular

View larger version (168K): [in this window] [in a new window]   Figure 11. At a higher magnification of the previous image, both myeloid and erythroid precursor are noted. The myeloid series is left shifted but distinct cytoplasmic granules noted on the aspirate are not apparent in this view.

View larger version (160K): [in this window] [in a new window]   Figure 12. The atypical large eosinophilic granules, however, are readily apparent at a higher magnification. There is a clustering of three such cells in the top center of this image and similar single cells are noted in other areas of the biopsy.

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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. Articles by McRae, B. Search for Related Content PubMed Articles by Lazarchick, J. Articles by McRae, B. Related Collections Phagocytic disorders - Chediak-Higashi, Pelger-Huet, May-Hegglin Related ASH-SAP Chapter Related Image Bank Image Sets Related ASH Education Book Article Social Bookmarking                   What's this?