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Rapidly fatal Klebsiella pneumoniae sepsis in a patient with pyruvate kinase deficiency and asplenia

Mohammad Faizan Zahid and Ashish Pal Singh Bains

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  • RE: Is non-transferrin bound iron (NTBI) a cofactor for the risk of post-splenectomy sepsis?
    • Tomas Ganz, Medicine and Pathology David Geffen School of Medicine at UCLA
    • Other Contributors:
      • Yonca Bulut, Pediatric Critical Care
      • Elizabeta Nemeth, Medicine

     

    We were intrigued by the blood smear from a patient with pyruvate kinase deficiency and asplenia, showing almost as many bacilli as erythrocytes, and foretelling his rapid death from sepsis, despite the administration of effective antibiotics. Fortunately, such catastrophes are rare among splenectomized patients1, raising the possibility of pathogenic cofactors that predispose to sepsis. Patients with erythrocyte pyruvate kinase deficiency suffer not only from hemolytic anemia but also from ineffective erythropoiesis2 resulting from excessive apoptosis of pyruvate kinase-deficient erythroblasts. Ineffective erythropoiesis suppresses the iron-regulatory hormone hepcidin3, leading to systemic iron overload even in patients who are not transfused. Iron overload is further exacerbated in those patients who require intermittent or regular erythrocyte transfusions. We suspect that the hepcidin suppression is mediated by high levels of erythroblast-generated erythroferrone, as is the case in β-thalassemia, a disease with overlapping pathogenesis. In turn, systemic iron overload causes increased plasma transferrin saturation and eventual generation of non-transferrin bound iron (NTBI). Our studies in mouse models indicate that NTBI is a potent enhancer of the growth of gram-negative bacteria including Klebsiella pneumoniae4,5, and that the pathogenicity of infection with these bacteria is dramatically enhance...

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    Conflict of Interest:
    None declared.