title: Medullary Microvascular Thrombosis and Injury in Sickle Hemoglobin C Disease.
abstract: Sickle cell nephropathy is a common complication in patients with sickle cell hemoglobinopathies. In these disorders, polymerization of mutated hemoglobin S results in deformation of red blood cells, which can cause endothelial cell injury in the kidney that may lead to thrombus formation when severe or manifest by multilayering of the basement membranes (glomerular and/or peritubular capillaries) in milder forms of injury. As the injury progresses, the subsequent ischemia, tubular dysfunction, and glomerular scarring can result in CKD or ESRD. Sickle cell nephropathy can occur in patients with homozygous hemoglobin SS or heterozygous hemoglobin S (hemoglobin SC, hemoglobin S/β(0)-thalassemia, and hemoglobin S/β(+)-thalassemia). Clinical manifestations resulting from hemoglobin S polymerization are often milder in patients with heterozygous hemoglobin S. These patients may not present with clinically apparent acute sickle cell crises, but these milder forms can provide a unique view of the kidney injury in sickle cell disease. Here, we report a patient with hemoglobin SC disease who showed peritubular capillary and vasa recta thrombi and capillary basement membrane alterations primarily involving the renal medulla. This patient highlights the vascular occlusion and endothelial cell injury in the medulla that contribute to sickle cell nephropathy.
authors.affiliation: Departments of Pathology and.
authors.forename: Mei Lin Z
authors.initials: ML
authors.lastname: Bissonnette
authors.affiliation: Departments of Pathology and.
authors.forename: Kammi J
authors.initials: KJ
authors.lastname: Henriksen
authors.affiliation: Nephrology, University of Chicago, Chicago, Illinois.
authors.forename: Kristie
authors.initials: K
authors.lastname: Delaney
authors.affiliation: Nephrology, University of Chicago, Chicago, Illinois.
authors.forename: Nicole
authors.initials: N
authors.lastname: Stankus
authors.affiliation: Departments of Pathology and anthony.chang@uchospitals.edu.
authors.forename: Anthony
authors.initials: A
authors.lastname: Chang
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications*; D001706:Biopsy; D005260:Female; D006445:Hemoglobin C Disease / Q000150:complications*; D006801:Humans; D007674:Kidney Diseases / Q000209:etiology*; D007679:Kidney Medulla / Q000098:blood supply* / Q000473:pathology; D055806:Microvessels; D012805:Sickle Cell Trait; D013927:Thrombosis / Q000209:etiology* / Q000473:pathology
keywords: endothelial cells; peritubular capillary; renal pathology; thrombosis