A case report of diffuse renal cortical necrosis in sickle cell trait

Abstract

Abstract Background: Sickle cell disorders are the most common hereditary hematological disorders; they are characterized by a genetic mutation at the ß-globin locus, the heterozygous inheritance leads to sickle cell trait (SCT), a largely benign phenotype with mild clinical manifestations, if any. Unlike sickle cell disease (SCD), clinical presentations for SCT patients are rarely attributed to their underlying hematological status. Different types of renal manifestations have been identified in SCT. Renal cortical necrosis (RCN) is a rare and severe form of kidney injury that has not been associated in the literature with SCT. We report a case of diffuse RCN with stage 3 of acute kidney injury in a patient with SCT with no apparent trigger and partial recovery. Case presentation: A 41-year-old non-smoker male, with a background of SCT and no other medical history presented with acute abdominal pain and an initial serum creatinine of 530 μmol/L. His toxicology, viral, and immunological screen were negative with a normal renal ultrasound scan. His urea was 14 mmol/L, hemoglobin of 12 g/dL, normal platelet levels and no schistocytes. His urine protein to creatinine ratio was 1040 mg/g. Lactate dehydrogenase (LDH) and total bilirubin were elevated at 2606 U/L and 31 umol/L, respectively. His kidney biopsy showed diffuse renal cortical necrosis. The patient received conservative therapy and had partial recovery of renal function; his creatinine improved to 176 micromol/L six months later with an eGFR of 42 ml/min/1.73m2 Conclusions: SCT has long been considered a benign condition. However, recent evidence suggests that, although uncommon, vaso-occlusive manifestations can occur, especially in the context of stressors such as dehydration or physical exhaustion. This is the first described case of diffuse RCN in SCT, highlighting the need for vigilance in managing SCT and its potential severe clinical manifestations.