A Case of Gross Hematuria in a 27-Year-Old Woman with Sickle Cell Disease During Pregnancy

Introduction: Sickle cell disease (SCD) is a genetic hematologic disorder characterized by the presence of hemoglobin S, leading to sickling of red blood cells and subsequent vaso-occlusion. In pregnancy, SCD presents unique challenges, as it can lead to complications like pain crises, renal issues, and hematuria. This case report presents a 27-year-old female with HbSC disease, who developed gross hematuria in the 20th week of pregnancy, along with a review of her clinical course, treatment, and outcome. Case Description: A 27-year-old pregnant woman (20 weeks gestation) with HbSC disease complicated by hip avascular necrosis and retinopathy presented with two days of gross hematuria. She denied dysuria, fever, or flank pain. Her only symptom on review of systems was bilateral leg pain from a prior sickle cell crisis. She reported no chronic NSAID use as an adult. Vital signs were stable: T 98.1°F, BP 98/63 mmHg, HR 78 bpm, RR 16. Labs showed normal renal function (Cr 0.47 mg/dL), mild pyuria, and significant hematuria (RBC >50/HPF). Urine protein-to-creatinine ratio was 1.53 g/g. CBC showed WBC 11.7K, Hgb 11.7, and Plt 293K. Renal ultrasound revealed normal kidney size and echogenicity without hydronephrosis or stones. The clinical diagnosis was papillary necrosis due to sickle cell nephropathy (SCN). Glomerulonephritis and UTI were considered less likely based on stable renal function, normal BP, and absence of urinary symptoms. She received an exchange transfusion and empiric ceftriaxone. The hematuria resolved by day 5 Discussion: Sickle cell nephropathy (SCN) results from renal vasculopathy, with cortical hyperperfusion and medullary hypoxia. It is most severe in HbSS and mildest in sickle cell trait. SCN leads to glomerular and tubular damage, reduced concentrating ability, and increased risk of papillary necrosis, hematuria, and infections. Proteinuria may be transient due to gross hematuria or signal underlying glomerulopathy. Gross hematuria from papillary necrosis is often self-limited but should prompt evaluation to rule out stones, urinary tract infection, or renal medullary carcinoma (RMC). Management includes hydration, rest, blood transfusion, antifibrinolytics like aminocaproic acid, and radical nephrectomy in refractory cases. RMC is a rare but aggressive malignancy seen in young patients with sickle hemoglobinopathies.