抄録
A 13 year-old student with severe anemia, hyperferremia and hepatomegaly well responded to pyridoxal-5-phosphate was described. Excellent hematological remission induced by the drug was confirmed on two occasions.
The hematological examination revealed marked, slightly hypochromic anemia with a morphological aberration of erythrocytes. Bone marrow picture indicated intense erythroid hyperplasia and maturation arrest with appearance of megaloblastoid erythroblasts (4.8%). Ringed sideroblasts and greatly increased iron stores in reticulum cells were also demonstrated.
The serum iron level was elevated and ferrokinetic study showed increased plasma iron turnover accompanied by decreased red cell utilization.
In the studies on heme synthesis, activity of δ-aminolevulinic acid sythetase was found to be greatly reduced. Increased excretion of xanthurenic acid in the urine was observed following administration of tryptophane.
A fall in the hemoglobin concentration recurred within two months when the treatment with pyridoxal-5-phosphate was interrupted. Readministration of the drug caused a marked reticulocytosis, an increase in the hemoglobin concentration and a decrease in the number of ringed sideroblasts in the marrow.
A possible role of pyridoxine on the development of pyridoxine responsive anemia was briely discussed.
