ÖMER EKİNCİ, ALİ DOĞAN, CENGİZ DEMİR

The presence of paroxysmal nocturnal hemoglobinuria in patients with idiopathic chronic renal failure

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal stem cell disease. Chronic renal f ailure (CRF) is defined as progressive deterioration in the regulation of fluid and electrolyte balance of the affected kidney due to a decrease in glomerular filtration rate resulting from various diseases. We also investigated the presence of PNH in foll ow-up patients diagnosed with end-stage renal failure (ESRD) of unknown etiology in this study. This study was carried out at the Yuzuncu Yil University in Van, Turkey. Sixteen patients with end-stage renal failure who had unknown etiology from a total of 143 patients were included in the study. The patients' age, gender, hemogram, biochemical parameters, and percentage of PNH clone were examined. PNH clone was analyzed by the Fluorescein Aerolysin method. Of the patients, 10 (62%) were female, 6 (38%) were male. Hemodialysis was performed in 15 patients (94%) as renal replacement therapy. Fourteen of the patients (88%) had anemia. LDH was elev ated in 6 patients (38%), and those with high LDH also suffered from anemia. PNH clone was negative for all patients as determined by FLAER analysis. A survey of the literature did not result in any study on the presence of paroxysmal nocturnal hemoglobinu ria in idiopathic chronic renal failure. This is the first such study with this focus. Based on review of the cases described in the literature , PNH should be considered in the differential diagnosis of renal failure cases which are unexplained and especia lly those accompanied by hemolysis findings.

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