AN INTERESTING CASE PIGMENT INDUCED ACUTE KIDNEYINJURY
Journal: University Journal of Medicine and Medical Specialities (Vol.2, No. 1)Publication Date: 2016-07-12
Authors : NOOR MOHAMED;
Page : 53-56
Keywords : paroxysmal nocturnal haemoglobinuria; phosphatidylinositol glycan; haemosiderin; acute kidney injury; clonal disorder; haemosiderin; hemolysis;
Abstract
AN INTERESTING CASE OF PIGMENT INDUCED ACUTE KIDNEYINJURYParoxysmal nocturnal haemoglobinuria (PNH) is an acquired clonal disorder associated with somatic mutations of the Xlinked phosphatidylinositol glycan anchor biosynthesis class A gene in haematopoietic stem cells, which results in the absence of the phosphatidylinositollinked proteins necessary to protect cells from complement-mediated lysis. The primary clinical manifestations of PNH include Intravascular haemolytic anaemia, thrombosis in vessels and bone marrow failure, which can cause pancytopenia. Reversible acute kidney injury (AKI) due to haemolysis-induced severe tubular damage is a recognized complication of paroxysmal nocturnal haemoglobinuria (PNH). It has been proposed that acute tubular necrosis and acute kidney injury associated with PNH results from tubular haemoglobin (Hb)-mediated toxicity due to haemolysis. We report a case of AKI associated to haemolysis. We report a case of AKI associated to PNH and extensive haemosiderin deposits in tubular cells which gave a positive reaction on Perls stain, consistent with deposition of ferric ions (haemosiderin).Patient was managed with intravenous fluids and on second day, there was normalization of serum creatinine. On follow-up patient continues To have normal serum creatinine
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Last modified: 2016-07-28 20:03:03