Copper Deficiency or Zinc Overload

The copper content of a Western diet averages 0.9 to 1.6 mg each day, which is only a few times greater than the amount needed to maintain homeostasis of this essential element. Copper deficiency has been described in malnourished premature infants, in patients receiving long-term parenteral or enteral hyperalimentation, after gastrectomy, with copper-chelating agents, or on an idiopathic basis. The syndrome of copper deficiency consists of sideroblastic anemia with hypochromic cells in the blood smear, accompanied by ring sideroblasts and vacuolated erythroid and myeloid precursors in the marrow, and of neutropenia with an absence of late myeloid forms in the marrow (Fig. 1). In some reports, patients present with neurologic symptoms such as paresthesias, weakness, or ataxia; and demyelination is seen on the magnetic resonance image of the brain. In infants, additional features may be seen, such as osteoporosis and long bone changes, depigmentation of skin and hair, and central nervous system abnormalities. The platelet counts remain normal. Serum copper and ceruloplasmin levels are low, whereas serum iron and transferrin saturation levels are normal. The serum zinc concentration may be increased. Prompt reversal of the hematologic changes follows therapy with 2 to 5 mg/day of copper sulfate taken orally or 100 to 500 μg/ day of copper supplement to the intravenous alimentation formula.

Fig1. This 70-year-old man was being treated with zinc supplementation and was found to have anemia and neutropenia (hemoglobin, 8.3 g/dL; hematocrit, 23.9%; and white blood cell count, 1200/µL). His peripheral smear (A) showed a biphasic erythroid population with some small slightly hypochromic cells and increased anisocytosis (red blood cell distribution width [RDW], 23.9%). The bone marrow aspirate showed a left shift in granulopoiesis with vacuolization of immature granulocytic and erythroid precursors (B, C). A Prussian blue–stained aspirate revealed ring sideroblasts (D). The patient’s copper level was less than 0.1 μg/mL (normal reference range, 0.75 to 1.45 μg/mL).

Large quantities of ingested zinc interfere with copper absorption and produce the neutropenia and sideroblastic anemia characteristic of copper deficiency. Zinc sulfate is freely available from health food stores, and as little as 450 mg/day for 2 years is sufficient for this effect. Sideroblastic anemia has also been ascribed to zinc toxicity arising from the ingestion of coins over a period of many years. Serum zinc levels are high, whereas serum copper and ceruloplasmin levels are low. Zinc must be discontinued for 9 to 12 weeks for full reversal of the anemia and neutropenia.

Iron Deficiency Anemia

In iron deficiency anemia, there is an accumulation of protoporphyrin in erythrocytes that rarely reaches the level found in EPP. The zinc complex of protoporphyrin is produced because FECH uses Zn2+ during iron-deficient erythropoiesis. Erythrocyte protoporphyrin may be raised before changes appear in peripheral blood and may be helpful in diagnosing iron deficiency when serum iron and ferritin levels are rising as a result of patients having started iron therapy. In iron-deficient erythropoiesis, erythroid ALAS activity is reduced below normal.

Hypothermia

Thrombocytopenia, erythroid hypoplasia, and ring sideroblasts have been described in patients with hypothermia associated with neuro logic disease. These changes reverse slowly as body temperature returns to normal.

Other Conditions

In hereditary tyrosinemia, excess urinary ALA is excreted because ALA dehydratase is inhibited by succinyl acetone. Like acute porphyria and lead poisoning, this disease is associated with neurobehavioral disturbance (see box on Lead Poisoning). In liver disease, there may be increased urinary excretion of coproporphyrin, predominantly isomer I. In the Dubin-Johnson syndrome, which is caused by mutations in the ABCC2 gene, the ratio of coproporphyrin isomer I to isomer III is markedly increased in the urine (>80%), possibly as a result of deficiency of hepatic uroporphyrinogen III cosynthase and increased activity of PBGD. In Rotor syndrome, total urinary excretion of coproporphyrin is markedly increased and consists predominantly of coproporphyrin isomer I. In the unconjugated hyperbilirubinemia of Gilbert syndrome, depressed activity of protoporphyrinogen oxidase and increased activity of ALAS has been found in peripheral leukocytes.

Environmental Intolerances

It has been hypothesized that several otherwise-unexplained chemical associated illnesses, such as multiple chemical sensitivity syndrome, may represent mild chronic cases of porphyria or other acquired abnormalities in heme synthesis. However, evidence for this concept is lacking.

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مواضيع ذات صلة

Morphologic Expression of Megaloblastosis

Sideroblastic Anemia and Porphyrinuria Caused by Drugs

Addison’s Disease

Cushing’s Disease

Sideroblastic anemias

Osteoporosis

Pathophysiology of Beta-Thalassemia Syndromes

Etiology and Pathophysiology of Anemia of Chronic Inflammation

Goitre and Thyroid nodules

Pathogenesis of Cobalamin Deficiency

Clinical Manifestations of Iron Overload

Pathobiology of Acquired Iron Overload