A 41-year-old male was referred for evaluation due to proteinuria.
Diagnosis of PNH in the context of hemolytic anemia.
She did not require blood transfusions and did not present any bleeding events.
Laboratory tests showed persistent hemolytic activity, elevated LDH (1,800-3,000 U/l) and undetectable haptoglobin, hemoglobin 8.5 mg/dl, normal leucopenia and mild thrombocytopenia, ferritin.
She had recurrent hemolytic crisis with fatigue, dysphagia, abdominal pain and dark urine.
The episodes used to last 1-3 days, every 2-3 months.
Renal function was normal, serum creatinine of 0.9 mg/dl and GFR (MDRD) > 60 ml/min. Persistent hemoglobinuria with normal urinary sediment, and proteinuria of 1.3 g/day with albuminuria 170 mg/day.
The acid-base, phosphate and acid-base status were normal.
The possibility of DTP due to hemosiderin deposit was considered, so a MRI was performed with the finding of bilateral renal cortical hypointensity compatible with iron deposit.
Therapy with eculizumab was initiated, but it was ruled out due to the lack of transfusion requirements and normal GFR.
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Two years later, she developed a severe hemolytic crisis with acute renal failure, which was attributed to hemoglobinuria.
The patient was treated with conservative measures, with recovery of renal function in 18 days.
After this episode, treatment with eculizumab was started.
In a few weeks, LDH and hemoglobin almost normalized, maintaining low levels of haptoglobin.
In the following months tubular proteinuria gradually returned to normal.
Fourteen months after starting eculizumab, a new MRI showed an improvement in signal hypointensity in the renal cortex of 34% and 51% in the left and right kidneys, respectively, indicating partial elimination of iron.
