Abstract

Copper is essential trace metal which plays vital role in electron transport reactions, iron hemostasis, pigment formation, neurotransmitter production, connective tissue biosynthesis and antioxidant defense mechanisms. A 58-year-old Caucasian man with ESRD on intermittent hemodialysis, chronic diarrhea and malabsorption after gastric bypass surgery on Intradialytic parenteral nutrition (IDPN) was evaluated for anemia with sub optimal response to high doses of erythropoietin and pancytopenia suspicious of myelodysplastic syndrome. Investigations listed in Figure 1. Bone Marrow biopsy showed refractory cytopenias with multi-lineage dysplasia. Cytogenetics showed no definitive immunophenotypic evidence of high-grade hematopoietic neoplasia, lymphoproliferative disease or plasma cell dyscrasia. He was started on daily oral therapy with copper gluconate with no improvement in the hematological parameters. Copper chloride was added to IDPN with improvement of all hematological parameters and copper levels [figure 2]. On attempting to withdraw therapy, severe drop in copper and cell counts were noted and was corrected with resumption of IDPN with copper supplementation. Copper deficiency can present as anemia and leukopenia, thrombocytopenia. Typical bone marrow findings mimic myelodysplastic syndrome including left shift in granulocytic & erythroid maturation & presence of ringed sideroblasts/hemosiderin containing plasma cells. Neurologic manifestations include ataxia, myeloneuropathy and cognitive deficits. Unlike neurologic deficits, hematologic manifestations can be reversed with copper supplementation. The current adult recommended intake is 0.9mg/day. The American society for metabolic and Bariatric surgery clinical practice recommend treatment of mild to moderate deficiency with oral (3-8mg/dl) and IV copper (2-4mg/dl) for severe deficiency. Oral supplementation may fail in patients with gastric bypass as copper is absorbed in stomach and duodenum. IDPN is relatively safe and efficacious modality of nutritional support which allows the administration of nutrients through extracorporeal circuit during hemodialysis in these patients. Other causes of copper deficiency include Post gastric bypass surgery, Excessive zinc ingestion or overloading during hemodialysis, celiac disease, cystic fibrosis, IBD and Bacterial overgrowth.2453_A Figure 1. Initial Patient Laboratory Values2453_B Figure 2. Hematological changes during treatment with copper. WBC - White blood cell count, HB - Hemoglobin, IDPN - Intradialytic Parenteral Nutrition, Oral - Oral supplementation

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