A 40-year-old male recently diagnosed with PNH for renal assessment.
In the previous 9 months he had presented repeated episodes of dark urine and low back pain.
He had severe hemolytic anemia, hemoglobin of 7.7 mg/dl, undetectable haptoglobin, elevated LDH 3,800 U/l, requiring transfusion.
Ferritin and saturation of transfer are normal.
Normal renal function, creatinine 0.8 mg/dl, proteinuria 427 mg/24 h, albuminuria 120 mg/24 h.
Hemoglobinuria with normal sediment, negative glycosuria and normal phosphate excretion.
In the following 10 months she presented repeated hemolytic crisis with macroscopic hemoglobinuria.
She reported difficulty concentrating and recurrent headache.
MRI showed marked signal hypointensity in the renal cortex in all sequences, and a brain MRI revealed multiple subcortical lacunar lesions.
Due to brain involvement attributable to PNH, treatment with eculizumab was initiated.
1.
The patient had hemoglobinuria and normalized LDH (persisting decreased haptoglobin).
Seventeen months after starting treatment, a new MRI showed normalization of the signal in the renal cortex.
Renal function remained normal, with proteinuria 130mg/day and negative hemoglobinuria.
