Abstract
We evaluated hematopoiesis in a 1 month-old infant with propionic acidemia who presented with pancytopenia. Her bone marrow (BM) showed dysmyelopoiesis and maturation arrest of all cell lines. Numerous mono- and multinucleated histiocytes engaged in hemophagocytosis were present. We studied the effects of the child's serum and of propionic acid (PA, pH 7.3) on mouse BM colony forming units-erythroid (CFU-E) and colony forming units-granulocytic-monocytic (CFU-GM) grown in fibrin clots. The patient's (pt's) serum significantly inhibited CFU-E but not CFU-GM compared to normal human sera. PA inhibited growth of CFU-E and CFU-GM as shown in the table. Semi-soft agar culture of human BM CFU-GM showed complete inhibition with 5 mM PA but none with 1 mM or 0.5 mM PA. There was no consistent inhibition of mouse or human BM cultures by glycine, tiglic acid, or 3-OH PA (1.0, 0.1, 0.01 mM, pH 7.3), all of which were elevated in this child. From these results we conclude that the pancytopenia and hematologic abnormalities found in this infant may be directly related to the effects of propionic acid.
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Stork, L., Ambruso, D., Wallner, S. et al. THE EFFECTS OF PROPIONIC ACIDEMIA ON HEMATOPOIESIS. Pediatr Res 18 (Suppl 4), 249 (1984). https://doi.org/10.1203/00006450-198404001-00939
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DOI: https://doi.org/10.1203/00006450-198404001-00939