Severe Pancytopenia due to Copper Deficiency

Ruocco, L; Baldi, Andrew; Cecconi, Nadia; Marini, Alessandra; Azzarà, Antonio; Ambrogi, F; Grassi, Bruno

doi:10.1159/000206060

Cited by 21 publications

(10 citation statements)

References 3 publications

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“…Dunlap et al (8) suggested that cytoplasmic vacuolization in early erythroid precursors is the first erythroid developmental change that occurs in cases of copper deficiency, because many patients with copper deficiency but without anemia exhibit this change. Most of the reported cases of copper deficiency (6,(8)(9)(10)(11)(12)(13)(14)(15), and all of the present copper-deficient subjects, exhibited cytoplasmic vacuolization of erythroid and myeloid precursors, which rapidly disappeared after copper supplementation. Although cytoplasmic vacuolization is also associated with other disorders, it may be particularly strongly associated with copper deficiency.…”

Section: Discussionmentioning

confidence: 64%

“…Copper deficiency has been observed in adult patients with inflammatory bowel disease (19,20), total parenteral nutrition (21,22), massive zinc ingestion (11) (6,(8)(9)(10)(11)(12)(13)(14)(15). Cytoplasmic vacuoles have also been found in patients with acute alcoholic intoxication (23), chloramphenicol toxicity (24), pancreatic dysfunction (25) and myeloproliferative syndromes (26).…”

Section: Discussionmentioning

confidence: 99%

“…Some such patients have anemia, often caused by iron deficiency due to gastrointestinal bleeding, but severe copper deficiency usually presents with anemia and or leucopenia (5)(6)(7). There have been reports of ringed sideroblasts and vacuolation of erythroid and myeloid precursors in patients with severe copper deficiency (6,(8)(9)(10)(11)(12)(13)(14)(15)…”

Section: Introductionmentioning

confidence: 99%

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Clinicopathological Analysis of Hematological Disorders in Tube-Fed Patients with Copper Deficiency

et al. 2007

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Section: Discussionmentioning

confidence: 64%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Clinicopathological Analysis of Hematological Disorders in Tube-Fed Patients with Copper Deficiency

et al. 2007

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“…In addition, sporadic case reports also suggested that inadequate diet alone or genetic disorders affecting proteins of copper metabolism could cause copper deficiency (28)(29)(30), including Menkes disease and occipital horn syndrome (10) …”

Section: Discussionmentioning

confidence: 99%

Cocoa Supplementation for Copper Deficiency Associated with Tube Feeding Nutrition

et al. 2006

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“…Less well characterized and more loosely associated were cytopenia and myeloneuropathy are signs and symptoms of copper deficiency were highlighted in several case reports. 1,2,[16][17][18][19][20] The most common causes of copper deficiency are malabsorption and malnutrition, but as noted above, copper deficiency can also occur secondary to chronic use of large doses of zinc causing zinc overload syndrome. 21,22 Responsive to copper supplementation or zinc withdrawal in cases the diagnosis of RARS should be avoided, since the anemia is not refractory to treatment and true myelodysplastic syndrome is not present.…”

confidence: 99%

Hypocupremia and bone marrow failure

et al. 2008

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Hypocupremia and bone marrow failureCopper deficiency associated with neurological disorders is a well-documented condition. However, hypocupremia is less often recognized as a cause of cytopenias or bone marrow failure. We report an illustrative series of three new cases of bi-lineage cytopenia associated with copper deficiency. We have analyzed clinical features of current and historical cases to identify clues that could facilitate application of appropriate laboratory testing and heighten the level of clinical suspicion. By maintaining an appropriately high level of suspicion for potential copper deficiency and obtaining a serum copper level, bone marrow failure due to this condition can be correctly diagnosed and treated. We suggest that copper deficiency be included in the differential diagnosis of reversible causes of bone marrow failure syndromes including myelodysplastic syndrome. 93:e1-e5 DOI: 10.3324/haematol.12121 IntroductionIn the evaluation of patients with pancytopenia, reversible causes of bone marrow failure such as nutritional deficiencies, viral infections, and medications need to be considered in the differential diagnosis. Copper deficiency can be easily treated but the diagnosis may be difficult due to comorbidities and low level of clinical suspicion.Patients with copper deficiency most commonly present with neurological disorders, including myeloneuropathy, arthralgias and neuropathy. [1][2][3][4] A diagnosis of copper deficiency is usually established by measuring serum copper or ceruloplasmin levels; a serum copper level <10 ∝mol/L or ceruloplasmin <180 mg/L is considered presumptive evidence of copper deficiency. The most common hematologic abnormalities associated with copper deficiency include anemia and neutropenia, while the platelet count usually remains normal.The adult diet in Western countries contains 0.6 to 1.6 mg of copper/d. Water, shellfish, organ meats, nuts, and grains are all sources of dietary copper. Absorption occurs through the intestinal mucosa across the basement membrane into the blood stream via energy dependent mechanisms where the availability and saturation of copper may limit absorption. Of significant interest is the physiologic relationship between copper and zinc. High zinc levels result in increased metallothionin production in enterocytes. The high affinity of copper to metallothionin displaces zinc and thus leads to the accumulation of copper in the enterocytes. The enterocytes are ultimately shed into the gastrointestinal tract, leading to copper elimination. 5 Copper plays a role as a cofactor for many enzymes, in electron-transporting proteins, and in antioxidant metabolism. The main copper-binding protein is ceruloplasmin which plays a role in copper transport and release and in protection of cells from oxidative damage. Both ferroxidase and ceruloplasmin may oxidize Fe 2+ to Fe 3+ facilitating the release of Fe from ferritin and its binding to transferrin. 6 Copper is required for a variety of enzymes, including amino oxidase, ferroxidase, c...

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Severe Pancytopenia due to Copper Deficiency

Cited by 21 publications

References 3 publications

Clinicopathological Analysis of Hematological Disorders in Tube-Fed Patients with Copper Deficiency

Clinicopathological Analysis of Hematological Disorders in Tube-Fed Patients with Copper Deficiency

Cocoa Supplementation for Copper Deficiency Associated with Tube Feeding Nutrition

Hypocupremia and bone marrow failure

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