Observations on the anemia and neutropenia of human copper deficiency.
Following extensive bowel resection, a young woman experienced severe malnutrition; subsequent administration of parenteral nutrition precipitated the copper deficiency syndrome. This consisted of hypocupremia, subnormal ceruloplasmin levels, anemia, and severe neutropenia. The bone marrow was megaloblastic, vacuolated, and sideroblastic; granulocytic maturation was not observed beyond the myelocyte stage. Copper sulfate therapy was followed by a marked reticulocytosis, increase in hematocrit, and recovery of neutrophils. Additional studies indicated that both serum and urinary erythropoietin values were low; serum activity increased after copper supplementation. Abnormal granulopoiesis was demonstrated using the in vitro granulocyte colony assay. The patient's granulcoytic stem cells were normal on two occasions; however, mixing studies showed that culture of the patient's copper-deficient marrow with her copper-deficient serum yielded significantly reduced numbers of granulocyte colonies. Thus, copper appears to be a necessary element for normal hematopoiesis; lack of this trace element may result in ineffective erythropoiesis and granulopoiesis.
D000328:Adult; D000380:Agranulocytosis / Q000150:complications*; D000740:Anemia / Q000150:complications*; D001854:Bone Marrow Cells; D002478:Cells, Cultured; D003115:Colony-Stimulating Factors / Q000494:pharmacology / Q000652:urine; D003300:Copper / Q000172:deficiency*; D004921:Erythropoietin / Q000097:blood / Q000652:urine; D005260:Female; D006410:Hematopoiesis; D006801:Humans; D009503:Neutropenia / Q000150:complications*
D003115:Colony-Stimulating Factors; D004921:Erythropoietin; D003300:Copper