Sickle cell trait and gross hematuria

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CASE PRESENTATION A 35-year-old African-American woman with sickle cell trait presented to our medical center with severe gross hematuria. The patient reported having had dark urine for at least 2 months, but after a recent 2 h and 30 min air travel, her urine became bright red and she began passing dark clots. In addition, she started experiencing generalized fatigue, weakness, and mild dizziness. The patient had experienced several episodes of gross hematuria in the past. One such episode, 15 years ago, resulted in a prolonged hospitalization and transfusion of 5 U of blood. Another episode took place 11 years ago lasting for approximately 2 weeks, but symptoms resolved spontaneously. The etiology of her hematuria had never been established. Other medical problems included chronic right knee osteoarthritis and degenerative lumbar disk disease. She denied chronic use of non-steroidal anti-inflammatory agents, but admitted to frequent use of acetaminophen for arthritic pain. Upon presentation, the patient appeared comfortable. Her blood pressure was 130/60, and heart rate was 80/min. Head, neck, lung, and heart examinations were unremarkable. The abdomen was soft and non-tender and liver span was normal. The tip of the spleen was palpable. There was no pedal edema. Her laboratory values were significant for mild anemia with hematocrit of 33% (normal 34-44%). The platelet count was 313 x 10 9 /l (normal 165-415 x 10 9 /l) and white blood cell count was 6.5 x 10 9 /l (normal 3.5-9.0 × 10 9 /l). Her coagulation parameters were normal. The serum electrolytes were also within normal range. Her renal function was preserved with a creatinine of 0.9 mg/dl (normal 0.5-0.9 mg/dl) and a blood urea nitrogen of 7 mg/dl (normal 7-20 mg/dl). Urine analysis revealed 3+ hemoglobin and no protein. Urine microscopy showed 182 red blood cells per high powerfield. Urine cultures were negative. Renal ultrasound measured the right kidney length at 11.7 cm; the left kidney was 11.9 cm in length; there was no hydronephrosis or calculus in either kidney. The urinary bladder was unremarkable. Magnetic resonance imaging confirmed that both kidneys were of normal size and signal, and no solid enhancing lesion was identified. Diagnostic cystoscopy and ureteroscopy were performed. Blood was visualized effluxing from the right ureteral orifice. Upon inspection of the collecting system, the posterior middle pole calyx was found to have a stenotic infundibulum. Endoinfundibulotomy was performed to enter the calyx, where there appeared to be a sloughed ischemic papilla with an accompanying hemorrhage (Figure 1). The papilla was fully fulgurated in an attempt to stop the bleeding. The remaining calyces and the renal pelvis were also inspected and found to be free of disease. Although the bleeding ceased in the immediate post-procedure period, it reappeared later that day.