Sickle cell trait and gross hematuria

Sickle cell trait, although mainly benign, may be associated with renal manifestations especially hematuria. Papillary necrosis is the most frequent cause of gross hematuria in patients with sickle cell trait. A complete work-up including renal imaging with ultrasound or CT scan should be performed in all sickle cell patients presenting with hematuria to evaluate for obstruction, kidney stones, tumors, and renal infarction. In young patients, CT or MR imaging is indicated to rule out RMC, a rare but deadly diagnosis associated with hematuria and sickle cell trait. Conservative measures should be first attempted when managing such patients. This includes bed rest, intravenous hydration, alkalinization of urine, and a blood transfusion if anemia is present. Ureterosopy with retrograde pyelography should be performed to confirm the diagnosis of renal papillary necrosis. A direct ureteroscopic fulgration or tamponade may be attempted at this time. If bleeding persists, desmopressin acetate (DDAVP®) and/or EACA (Amicar®) infusion can be attempted as a next step in management. In our opinion, there is insufficient experimental data to support the use of oral or intravenous urea. For refractory cases, renal angiography with embolization may be attempted, although simultaneous angiography and ureteroscopy has been reported to be more successful.