Hematologic Manifestations of Copper Deficiency: One Year Experience of a Single Institution
N Cetin, K Oza, Z Singh, J Bornhorst, L Chen, R Lorsbach. University of Arkansas for Medical Sciences, Little Rock, AR; UT Medical School, Houston, TX
Background: Copper deficiency can cause pancytopenia and dyserythropoesis in the bone marrow (BM) mimicking myelodysplastic syndrome (MDS). After copper supplementation, hematological parameters rapidly improve. However, copper levels are not routinely checked in patients with cytopenias. The literature on the hematological manifestations of copper deficiency and its awareness amongst clinicians is limited. The purpose of this study was to investigate the prevalence, laboratory and hematological manifestations of copper deficiency.
Design: The laboratory database at UAMS was searched for patients with a serum copper determination in the year 2005. Patients with low copper levels (<80mcg/dL; normal range 85-155mcg/dL) were selected for further analysis. The hematologic data and BM aspirates were reviewed and correlated with clinical characteristics and laboratory parameters.
Results: A total of 185 tests for serum copper were performed. Forty patients (22%) with hypocupremia were identified; the primary diseases were: cirrhosis 13, malignancies 8, gastric surgery 7 neurological diseases 4, nutritional deficiencies 2, diabetes 2, autoimmune 2, cystic fibrosis 1, and familial polyposis 1. Laboratory studies revealed decreased white blood cell (WBC) count, hematocrit, and platelet counts in 19, 26, and 27 patients respectively. The mean corpuscular volume was decreased in 4/40 and increased in 7/40 patients. Serum ceruloplasmin level was low in 3/17. High serum zinc level was observed in 4/17 patients. BM evaluation performed in 6 patients typically documented dyserythropoesis with erythroid vacuolation, ringed sideroblasts, and presence of iron within plasma cells. In 2 patients the trilineage dyspoesis was significantly pronounced so as to mimic primary MDS. Hemophagocytosis and marrow hypoplasia were noted in one case each.
Conclusions: Our findings demonstrate that copper deficiency frequently manifests as clinically significant peripheral blood cytopenias and is often associated with dyserythropoiesis. This study highlights the importance of evaluating serum copper level to exclude hypocupremia as the cause in cytopenic patients to avoid misinterpretation of the peripheral blood and BM findings as indicative of primary MDS. A high index of suspicion for copper deficiency is particularly warranted in patients with liver disease, in those who have undergone gastrectomy, or in the presence of typical BM findings as described above.
Wednesday, March 11, 2009 1:00 PM
Poster Session VI # 206, Wednesday Afternoon