'Myelokathexis'. Neutropenia with marrow hyperplasia
O'regan, S.; Newman, A.J.; Graham, R.C.
American Journal of Diseases of Children 131(6): 655-658
1977
ISSN/ISBN: 0002-922X PMID: 868817 Document Number: 122301
A 10 yr old girl was 1st seen at age 5 yr with pneumonia and neutropenia. Since then, she has remained leukopenic, although manifesting a leukocytosis only when she has pulmonary infection. A rapid fall in her peripheral WBC count occurs with initiation of antibiotic therapy. Despite her neutropenia, marked myeloid hyperplasia is evident on marrow smear examination; many cells being hypersegmented with fine intralobular bridging with chromatin strands and cytoplasmic vacuolation. The peripheral WBC response to epinephrine administration did not indicate a shift from the circulating to marginal neutrophil pool. Results from a Rebuck skin window test suggested poor neutrophil tissue mitration. A defect in granulocyte release from the patient's marrow may explain these bizarre hematologic findings.