A 32-year-old man with active
Crohn's disease and recurrent small bowel
strictures underwent abdominal surgery and was subsequently given
total parenteral nutrition (TPN). Severe
cholestasis developed and
copper was removed from the TPN. Although serum
ceruloplasmin levels were within normal limits, 8 weeks after
copper removal, he developed
pancytopenia. Serum
copper levels were severely depressed. Bone marrow biopsy was consistent with
copper deficiency; cytoplasmic vacuolization of both myeloid and erythroid precursors, megaloblastic erthropoiesis, and marked hypocellularity were observed. IV replacement with
copper sulfate resulted in improvement in the patient's
anemia,
neutropenia, and
thrombocytopenia, but the patient died suddenly from
cardiac tamponade. Postmortem examination revealed fibrinous and hemorrhagic
pericarditis. Despite the rare occurrence of overt
copper deficiency, this case emphasizes the need to recognize
copper deficiency as an important etiology of
iron-resistant
anemia in patients receiving TPN. Furthermore, the relative rapidity with which our patient developed
pancytopenia suggests that, in view of the established recommendation that
copper be removed from TPN in cholestatic conditions, serum
copper levels must be measured periodically.