Paroxysmal nocturnal hemoglobinuria complicated with chronic tubulointerstitial nephritis: a case report and literature review / 中华肾脏病杂志

ABSTRACT

Objective:To report a rare case of paroxysmal nocturnal hemoglobinuria (PNH) complicated with chronic tubulointerstitial nephropathy, combined with literature review, and discuss the clinical, imaging and pathological characteristics of the disease and the diagnosis and treatment ideas.Methods:The patient's clinical data, magnetic resonance imaging (MRI) and kidney pathological examination results, treatment measures and effects were collected and reported. Through systematic review of relevant literature, the clinical manifestations and pathogenesis of chronic tubular interstitial nephropathy complicated by PNH were summarized and discussed.Results:In this case, PNH was diagnosed for more than 30 years, the peripheral blood PNH clone was positive, urine specific gravity was 1.012, urine pH 6.0-7.0, urine protein (+), urine sugar (3+), serum creatinine 259 μmol/L, serum lactic acid dehydrogenase 800 U/L. MRI showed bilateral renal cortical signal was low intensity on both T1- and T2- weighted images. Kidney biopsy revealed remarkable chronic tubulointerstitial nephropathy with massive hemosiderin deposition in proximal tubular cells demonstrated by Prussian blue staining and electron microscopy. By using low-dose prednisone to control hemolytic attack and other supportive treatments, the patient's renal function has been stabilized for a long time.Conclusions:PNH complicated with chronic tubulointerstitial nephritis is easy to be misdiagnosed due to insidious onset. MRI and kidney histopathological examination are helpful to clarify the diagnosis. Early diagnosis and treatment are helpful to improve the prognosis of such patients.