Paroxysmal nocturnal haemoglobinuria presented as acute kidney injury
DOI:
https://doi.org/10.18203/2320-6012.ijrms20261360Keywords:
Acute kidney injury, Flow cytometry, Paroxysmal nocturnal hemoglobinuriaAbstract
Paroxysmal nocturnal hemoglobinuria (PNH) is rare disease, caused by acquired somatic mutations in PIG-A gene. Renal involvement in PNH varies from reversible acute kidney injury (AKI) to chronic irreversible damage. We are presenting a case of PNH in young female who presented as AKI. Final diagnosis was made by flowcytometry which showed evidence of PNH clone based upon analysis of a variety of GPI linked antibodies (FLAER, CD59) on monocytes, granulocytes and RBCs. PNH presenting as recurrent acute renal failure is extremely rare. We reported this case to highlight a rare, but potentially reversible cause of acute renal failure. These types of cases need high index of suspicion. Early diagnosis and treatment will help in preventing repeated episodes of AKI and thus chronic kidney disease.
References
Pokhrel B, Gautam S, Khanal S, Pokhrel NB, Shrestha A. A Rare and Misdiagnosed Entity Paroxysmal Nocturnal Hemoglobinuria: A Case Report. Cureus. 2021;13(5):e14902.
Macedo ES, Filho SLAP, Pro JDZ, Rolim VDM, Primo GDAS, Brunetta DM, et al. Renal involvement in paroxysmal nocturnal hemoglobinuria: a brief review of the literature. Rev Assoc Med Bras. 2018;64(12):1139-46.
Balları J, Arce Y, Balcells RT, Encarnacio MD, Manzarbeitia F, Ortiz A, et al. Acute renal failure associated to paroxysmal nocturnal haemoglobinuria leads to intratubular haemosiderin accumulation and CD163 expression. Nephrol Dial Transplant. 2011;26(10):3408-11.
Chen SC, Hung CC, Hsu CP, Chiu YW, Liu YC, Tsai JC, et al. Recurrent acute renal failure in a patient with aplastic anemia-paroxysmal nocturnal hemoglobinuria syndrome: a case report. Kaohsiung J med Sci. 2007;23(11):579-83.
Hussain S, Qureshi A, Kazi J. Renal Involvement in Paroxysmal Nocturnal Hemoglobinuria. Nephron Clin Pract. 2013;123(2):28-35.
Balwani MR, Kute VB, Shah PR, Shah M, Varyani U, Trivedi HL. Manifestation of paroxysmal nocturnal hemoglobinuria as repeated acute kidney injury. J Nephropharmacol. 2016;5(2):116-8.
Nair RK, Khaira A, Sharma A, Mahajan S, Dinda AK. Spectrum of renal involvement in paroxysmal nocturnal hemoglobinuria: report of three cases and a brief review of the literature. Int Urol Nephrol. 2008;40(2):471-5.
Puri V, Gandhi A, Sharma S. Renal Biopsy in Paroxysmal Nocturnal Hemoglobinuria: An Insight into the Spectrum of Morphologic Changes. Indian J Nephrol. 2017;27(4):284-8.
Kokoris SI, Gavriilaki E, Miari A, Travlou A, Kyriakou E, Anagnostopoulos A, et al. Renal involvement in paroxysmal nocturnal hemoglobinuria: an update on clinical features, pathophysiology and treatment. Hematology. 2018;23(8):558-66.
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