Pancytopenia after removal of copper from total parenteral nutrition

Patients who develop cholestatic jaundice during chronic total parenteral n utrition (TPN) can develop significant hematologic complications due to hyp ocupremia if copper supplementation is withheld. A 36 year-old female with short bowel syndrome developed progressive liver dysfunction 6 months after initiation of TPN. Trace elements were omitted from her TPN because of cho lestasis and persistent hyperbilirubinemia. Despite chronic diarrhea, absor ption of some dietary copper was anticipated from her oral diet. Fifteen mo nths later, the patient became red cell transfusion dependent, and her neut rophil and platelet counts steadily declined. After 19 months of receiving TPN without trace elements, her serum copper level was 25 mug/dL (normal: 7 0 to 155 mug/dL). Provision of trace elements for 2 months was associated w ith increased serum copper, neutrophil and platelet counts and independence from red cell transfusions. When the serum copper level reached 186 mug/dL , copper supplementation was discontinued. Over the next 3 months, serum co pper level fell to 10 mug/dL, neutrophil and platelet counts fell precipito usly, and red cell transfusions were resumed. Once again, copper, neutrophi l and platelet levels promptly rebounded with parenteral copper supplementa tion. Although anemia and neutropenia are well-recognized hematologic conse quences of copper deficiency, thrombocytopenia rarely has been reported. Th is is the first report of pancytopenia secondary to TPN-related copper defi ciency in which the association was confirmed when hypocupremia recurred.