Abstract
A 13 year-old male presented with recurrent cellulitis and osteomyelitis and persistent neutropenia (blood neutrophils count of 100/mm3). The bone marrow was normal except for two nuclei in the majority of metamyelocytes and bands and in all segmented neutrophils. No binucleated myeloblasts, myelocytes or other cell types were found. Granulocyte-monocyte colonies cultured from bone marrow had single nucleated and binucleated band forms and metamyelocytes, but no binucleated myelocytes or myeloblasts. Cytogenetic studies of bone marrow cells showed that the single nucleated cells were 46XY, while the binuclear cells were predominantly 92XXYY.
Chemotaxis of myeloid bone marrow cells was examined by a subagarose method. The patient's cells showed no directed movement towards zymogan activated serum (ZAS) or a synthetic peptide, F-Met-Phe, whereas directed and random movement of myeloid bone marrow cells from adult controls was detected (F-met-Phe, 13.2 u/mm and 9.5 u/min; ZAS 22 u/min and 11.7 u/min).
These findings suggest that defective egress of binucleated tetraploid neutrophils from the bone marrow resulted in chronic neutropenia. Further studies on the cytoplasmic architecture of these cells may help define a link between the failure of cytoplasmic splitting and the impaired chemotaxis of myeloid cells.
Log in or create a free account to read this content
Gain free access to this article, as well as selected content from this journal and more on nature.com
or
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Mamlok, R., Juneja, H., Elder, F. et al. NEUTROPENIA AND DEFECTIVE CHEMOTAXIS ASSOCIATED WITH BINUCLEAR, TETRAPLOID MYELOID-MONOCYTIC LEUKOCYTES. Pediatr Res 21 (Suppl 4), 314 (1987). https://doi.org/10.1203/00006450-198704010-00882
Issue date:
DOI: https://doi.org/10.1203/00006450-198704010-00882
