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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 Neutropenia/agranulocytosis; PB, BM - several variants - absent myelopoeisis, myelocyte "arrest" Related ASH-SAP Chapter Related Image Bank Image Sets Related ASH Education Book Article Social Bookmarking                   What's this?

Kostmann's Syndrome

View larger version (181K): [in this window] [in a new window]   Figure 1. The patient is a 10 month old Caucasian male with a history of recurrent upper respiratory infections and otitis media since birth. His CBC at this time included a H/H of 10.9g/dl/32.4%, platelet count of 280,000/ul and a WBC of 3840/ul with a differential of 25% monocytes, 70% lymphocytes, 4% eosinphils, 1% basophils and 1% neutrophils. His ANC was 38/ul. After an extensive evaluation he was diagnosed as having congenital neutropenia consistent with Kostmann's syndrome. The peripheral smear confirmed the leukopenia and almost complete absence of neuutrophils. A single hyposegmented PMN can be seen in this view.

View larger version (138K): [in this window] [in a new window]   Figure 2. A touch preparation done on the marrow biopsy shows a marked increase in myeloid precursors without evidence of maturation. Most myeloid cells present are either at the promyelocytic or myelocytic stages of maturation. In addition, eosinphils are increased. There is also an increase in small to intermediate sized lymphocytes.

View larger version (144K): [in this window] [in a new window]   Figure 3. A low power view of the aspirate shows an increase in lymphocytes and scattered erythroid precursors. Myeloid precursors (blasts, promyelocytes, and myelocytes) can readily be seen in this view but there is no evidence of maturation in the myeloid series.

View larger version (125K): [in this window] [in a new window]   Figure 4. In this view, erythroid precursors and eosinophils are prominent. Note also several basophils. Myeloid maturation is absent. The prominent lymphocytosis is again seen. Many of these cells have the morphologic appearance of hematogones.

View larger version (129K): [in this window] [in a new window]   Figure 5. The cellular spectrum in this view is similar to that seen on the previous image, however, there is in addition a notable increase in eosinophilic myelocytes.

View larger version (164K): [in this window] [in a new window]   Figure 6. The cellularity of the biopsy is approximately 85%. Normal numbers of megakaryocytes can be seen scattered throughout the biopsy. Many small "blue" cells are noted.

View larger version (155K): [in this window] [in a new window]   Figure 7. The prominent myeloid hyperplasia is best seen in this view. There are >10 rows of myeloid precursors seen in the paratrabecular area but no terminal myeloid maturation. Normally myeloid cells in this region are 1-3 cells deep.

View larger version (151K): [in this window] [in a new window]   Figure 8. A closer view of another paratrabecular area shows a marked proliferartion of eosinophilic precursors and mature eosinophils.

View larger version (155K): [in this window] [in a new window]   Figure 9. A CD3 immunostain of the marrow shows a slight increase of T-cells scattered throughout the biopsy.

View larger version (164K): [in this window] [in a new window]   Figure 10. In contrast, a CD20 immunostain shows that the B-cells are markedly increased.

View larger version (154K): [in this window] [in a new window]   Figure 11. Most of these cells were also CD10 positive on immunostaining. The dual CD20/CD10 positivity of the these cells is consistent with these lymphocytes being hematogones, Immunophenotyping of the marrow by flow cytometry confirmed this finding.

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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 Neutropenia/agranulocytosis; PB, BM - several variants - absent myelopoeisis, myelocyte "arrest" Related ASH-SAP Chapter Related Image Bank Image Sets Related ASH Education Book Article Social Bookmarking                   What's this?