Acute kidney injury with dark urine: the case of paroxysmal nocturnal hemoglobinuria
In this case report; we describe a patient with severe attack of paroxysmal nocturnal hemoglobinuria (PNH) following Ciprofloxacin therapy. He presented with recurrent abdominal pain, repeated vomiting and dark urine. Physical examination revealed severe pallor and jaundice. Laboratory investigations showed severe intravascular hemolysis with negative Coomb’s test, progressive acute kidney injury (AKI), sterile blood cultures and negative serology for autoimmune diseases. Subsequently, he developed pancytopenia. Ham test was positive and flow cytometry, later on, confirmed PNH. He was supported with multiple transfusions of packed blood cells and hemodialysis. Ciprofloxacin was discontinued and his PNH was treated with Solumedrol 1 g daily for 3 days followed by Prednisone 60 mg/day for 2 months. Two weeks later; his hemolysis abated and his AKI improved. Up to 5 years later, he still has minor PNH clone yet without disease activity. In conclusion; our patient had acute drug-induced hemolytic crisis associated with minor PNH clone. With drug-vigilance; no further relapses were reported and his PNH clone remained stable for 5 years. The case expands the spectrum of PNH phenotypes and its triggering factors.
Keywords: AKI, anemia, autoimmune, flow cytometry, hemolysis, PNH.
2- Medof ME, Lublin DM, Holers VM, Ayers DJ, Getty RR, Leykam JF, Atkinson JP, Tykocinski ML. Cloning and characterization of cDNAs encoding the complete sequence of decay-accelerating factor of human complement. Proc Natl Acad Sci USA 1987; 84:2007-2011.
3- Parker C, Omine M, Richards S, et al. Diagnosis and management of paroxysmal nocturnal hemoglobinuria. Blood 2005; 106:3699-3709.
4- Hill A, DeZern AE, Kinoshita T, Brodsky RA. Paroxysmal nocturnal haemoglobinuria. Nat Rev Dis Primers. 2017 May 18. 3:17028.
5- Mehta RL, Kellum JA, Shah SV, et al. Acute Kidney Injury Network: report of an initiative to improve outcomes in acute kidney injury. Crit Care 2007; 11:R31.
6- Kidney Disease: Improving Global Outcomes (KDIGO) Acute Kidney Injury Work Group. KDIGO clinical practice guideline for acute kidney injury. Kidney Int 2012; 2(suppl):1-138.
7- Ranganath LR, Jarvis JC, Gallagher JA. "Recent advances in management of alkaptonuria (invited review; best practice article)". J Clin Pathol 2013; 66:367–73.
8- Capriotti, Theresa (2016). Pathophysiology: introductory concepts and clinical perspectives. Frizzell, Joan Parker. Philadelphia.
9- Elise S, Michael Halefom M. The clinical significance of schistocytes: A prospective evaluation of the international councel for standerdiation in hematology schistocytes guidline. Turk J Hematol 2017; 34:59-63.
10- Dvanajscak Z, Walker PD, Cossey LN, Messias NC, Boils CL, Kuperman MB, Larsen CP. Hemolysis-associated hemoglobin cast nephropathy results from a range of clinicopathologic disorders. Kidney Int 2019; 96:1400-1407.
11- Fletcher M, Sutherland DR, Whitby L, Whitby A, Richards SJ, et al. Standardizing leucocyte PNH clone detection: An international study. Cytometry Part B 2014; 86B:311-318.
12- Nesargikar PN, Spiller B, Chavez R. The complement system: history, pathways, cascade and inhibitors. Euro J Microbiol Immunol 2012; 2:103-111.
13- Rother RP, Bell L, Hillmen P, Gladwin MT. "The clinical sequelae of intravascular hemolysis and extracellular plasma hemoglobin: a novel mechanism of human disease". JAMA 2005; 293:1653–1662.
14- Patriquin CJ, Kiss T, Caplan S, Chin-Yee I, Grewal K, et al. How we treat paroxysmal nocturnal hemoglobinuria: A consensus statement of the Canadian PNH network and review of the national registry. Euro J Hematol 2018; 102:36-52.
15- van den Heuvel-Eibrink MM, Bredius RG, te Winkel ML, et al. Childhood paroxysmal nocturnal haemoglobinuria (PNH), a report of 11 cases in the Netherlands. Br J Haematol 2005; 128:571-577.
16- Tichelli A, Socie G, Marsh J, et al. Outcome of pregnancy and disease course among women with aplastic anemia treated with immunosuppression. Ann Intern Med 2002; 137:164-172.
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