Anemia and Neutropenia in a Case of Copper Deficiency: Role of Copper in Normal Hematopoiesis

Abstract

We present a patient who developed severe anemia and neutropenia after receiving parenteral nutrition for 2.5 years. The serum levels of copper and ceruloplasmin were low, and the bone marrow showed the presence of ringed sideroblasts and vacuolated immature cells. The administration of copper chloride by bolus injection led to a rapid improvement in anemia and neutropenia. The number of progenitor cells (colony-forming unit-granulocyte-macrophage and erythrocyte) present before the copper supplementation was well preserved. It is therefore suggested that copper enzymes play an important role in the maturation of hematopoietic cells.

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

“…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).…”

“…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)…”

Engineering cord blood to improve engraftment after cord blood transplant

“…Severe anemia and neutropenia are well-recognized signs of copper deficiency, resulting from ineffective erythropoiesis and granulopoiesis due to the arrest of progenitor cell maturation (45)(46)(47). Similar effects can be induced by using a high affinity copper chelator which decreases the intracellular copper content (48 had engraftment from the expanded cord at the time of engraftment while the rest engrafted from the unmanipulated unit.…”

Correction of Copper Deficiency Improves Erythropoietin Unresponsiveness in Hemodialysis Patients with Anemia

“…Mitochondria isolated from copper-deficient animals were lacking in cytochrome oxidase activity and failed to synthesize heme from ferric iron and protoporphyrin at the normal rate, perhaps leading to mitochondrial iron accumulation (11). In addition, copper/zinc superoxide dismutase (Cu/Zn-SOD), an antioxidant enzyme, activity is decreased to 20% of the normal erythrocyte Cu/Zn-SOD in the copper-deficient subject, which may accelerate disposision of superoxide and shorten the life span of erythrocytes (12).…”