Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027947 (neutropenia)
 17,527 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Patients who develop cholestatic jaundice during chronic total parenteral nutrition (TPN) can develop significant hematologic complications due to hypocupremia 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 cholestasis and persistent hyperbilirubinemia. Despite chronic diarrhea, absorption of some dietary copper was anticipated from her oral diet. Fifteen months later, the patient became red cell transfusion dependent, and her neutrophil and platelet counts steadily declined. After 19 months of receiving TPN without trace elements, her serum copper level was 25 microLg/dL (normal: 70 to 155 microg/dL). Provision of trace elements for 2 months was associated with increased serum copper, neutrophil and platelet counts and independence from red cell transfusions. When the serum copper level reached 186 microg/dL, copper supplementation was discontinued. Over the next 3 months, serum copper level fell to 10 microg/dL, neutrophil and platelet counts fell precipitously, and red cell transfusions were resumed. Once again, copper, neutrophil and platelet levels promptly rebounded with parenteral copper supplementation. Although anemia and neutropenia are well-recognized hematologic consequences of copper deficiency, thrombocytopenia rarely has been reported. This is the first report of pancytopenia secondary to TPN-related copper deficiency in which the association was confirmed when hypocupremia recurred.
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PMID:Pancytopenia after removal of copper from total parenteral nutrition. 1107 96

We present a case of pancytopenia in a 9-month-old infant with total parenteral nutrition (TPN) dependence due to short bowel syndrome. Bone marrow examination revealed left-shifted myeloid maturation, erythroid and myeloid dysplasia with normal iron stores. Serum copper level was 2 microm/dl (normal range 90-190 mcg/dl). After supplementation, copper levels normalized at 143 mcg/dl, and the macrocytic anemia, neutropenia, and thrombocytopenia resolved. Copper deficiency should be considered in the differential diagnosis of cytopenias and myelodsyplasia, particularly in the growing number of pediatric patients with TPN dependency or malabsorption.
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PMID:Pancytopenia with myelodysplasia due to copper deficiency. 1862 12

Copper deficiency is an uncommon, but treatable cause of hematologic abnormalities. We present and describe two interesting cases in this report. The first case was a 37-year-old man with history of short bowel syndrome and long-term total parenteral nutrition (TPN) presenting with pancytopenia and chronic symmetrical polyarthritis that resembled rheumatoid arthritis. The second case was a 64-year-old man with malabsorption from Cronkhite-Canada Syndrome (CCS) and history of subtotal gastrectomy presenting with macrocytic anemia and neutropenia. Bone marrow examination in both cases revealed cytoplasmic vacuolization of myeloid and erythroid precursors. After copper supplementation was initiated, hematological abnormalities and arthritis were significantly improved. We encourage clinicians to recognize early and identify copper deficiency in patients who have unexplained cytopenia, especially if there is history of upper gastrointestinal tract surgery, malabsorption, or long-term TPN.
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PMID:Anemia and Neutropenia in Copper-Deficient Patients: A Report of Two Cases and Literature Review. 2990 25