Energy-dispersive X-ray analysis of the mitochondria of sideroblastic anaemia.
Energy-dispersive X-ray analysis has been performed on marrow sideroblasts obtained from 10 patients with sideroblastic anaemias or erythroleukaemia (six primary refractory sideroblastic anaemia, two pyridoxine-responsive, one secondary sideroblastic anaemia, two erythroleukaemia). Irrespective of the nature of the disorder associated with the presence of sideroblasts, X-ray analysis of siderotic mitochondria consistently revealed the presence of iron and phosphorus with the average Fe/P intensity ratio measuring 1.4-1.5. Other elements variably detected within siderotic mitochondria included calcium, lead, potassium and zinc. Variation in the presence of these latter elements was detected not only between different patients, but also within different samples taken at different times from a single patient and even among different cells of the same sample. Despite the detection of lead in siderotic mitochondria of a significant number of patients (five out of seven), there was no clinical evidence of lead toxicity. The elemental composition of the intramitochondrial deposits in sideroblasts was distinct from that of ferritin or haemosiderin and probably consists of ferric phosphate, possibly, ferric orthophosphate (FePO4).
D000756:Anemia, Sideroblastic / Q000378:metabolism*; D001853:Bone Marrow / Q000378:metabolism*; D004577:Electron Probe Microanalysis; D005293:Ferritins / Q000378:metabolism; D006485:Hemosiderin / Q000378:metabolism; D006801:Humans; D007501:Iron / Q000378:metabolism; D004915:Leukemia, Erythroblastic, Acute / Q000378:metabolism; D008928:Mitochondria / Q000378:metabolism; D010758:Phosphorus / Q000378:metabolism
D010758:Phosphorus; D005293:Ferritins; D006485:Hemosiderin; D007501:Iron