title: Severe anemia and neutropenia associated with hyperzincemia and hypercalprotectinemia.
abstract: Calprotectin, also known as the S100A8/A9 or MRP8/14 complex, is a major calcium-binding protein in the cytosol of neutrophils, monocytes, and keratinocytes. It differs from other S100 proteins in its zinc-binding capacity. The authors describe a 4-year-old girl with severe anemia, neutropenia, inflammation, and severe growth failure. Bone marrow examination showed moderate dyserythropoiesis. No hemolysis, iron deficiency, hemoglobinopathies, immunologic diseases, or autoantibodies were detected. Serum levels of copper and ceruloplasmin were within the normal range, although the serum zinc concentration was markedly increased (310 microg/dL). Urinary zinc excretion and erythrocyte zinc concentrations were within the normal range. Family studies showed normal zinc and copper plasma levels. The patient's plasma calprotectin concentration showed a 6,000-fold increase (2,900 mg/L) compared with normal values. The calprotectin concentration is known to be elevated in many inflammatory conditions but is generally below 10 mg/L and thus far below the levels reported in this patient. The authors describe this case as an inborn error of zinc metabolism caused by dysregulation of calprotectin metabolism, which mainly presented with the features of microcytic anemia and inflammation.
authors.affiliation: Second Department of Pediatrics, P & A Kyriakou Children's Hospital, Athens,Greece.
authors.forename: Smaragdi
authors.initials: S
authors.lastname: Fessatou
authors.forename: Magne K
authors.initials: MK
authors.lastname: Fagerhol
authors.forename: Johannes
authors.initials: J
authors.lastname: Roth
authors.forename: Alexandra
authors.initials: A
authors.lastname: Stamoulakatou
authors.forename: Vassiliki
authors.initials: V
authors.lastname: Kitra
authors.forename: Michaela
authors.initials: M
authors.lastname: Hadarean
authors.forename: George
authors.initials: G
authors.lastname: Paleologos
authors.forename: Helen
authors.initials: H
authors.lastname: Chandrinou
authors.forename: Barry
authors.initials: B
authors.lastname: Sampson
authors.forename: Ioannis
authors.initials: I
authors.lastname: Papassotiriou
mesh_terms: D000740:Anemia / Q000097:blood* / Q000209:etiology; D002675:Child, Preschool; D005260:Female; D006130:Growth Disorders / Q000097:blood / Q000209:etiology; D006801:Humans; D039841:Leukocyte L1 Antigen Complex / Q000097:blood*; D008661:Metabolism, Inborn Errors / Q000097:blood* / Q000150:complications / Q000628:therapy; D009503:Neutropenia / Q000097:blood* / Q000209:etiology; D015032:Zinc / Q000097:blood*
chemical_list: D039841:Leukocyte L1 Antigen Complex; D015032:Zinc