Autoinflammatory syndrome with cytopenia, hyperzincemia, and hypercalprotectinemia (AICZC) is characterized by chronic systemic inflammation, prominent skin inflammation, arthralgias/arthritis, hepatosplenomegaly, pancytopenia, and failure to thrive. A hallmark of the disease is the extreme increase in serum concentrations of zinc and the proinflammatory alarmins MRP8 (S100A8; 123885) and MRP14 (S100A9; 123886), which make up the heterodimeric calcium- and zinc-binding protein complex calprotectin. Severe anemia and neutropenia are accompanied by thrombocytopenia in most patients, and bone marrow aspirates show dysgranulopoiesis and dyserythropoiesis. Intrafamilial variable expressivity and incomplete penetrance have been observed (summary by Holzinger et al., 2015).