eISSN: 2345-4202  
J Nephropharmacol. 2016;5(2):116-118.
PMID: 28197517
PMCID: PMC5297565

Case Report

Manifestation of paroxysmal nocturnal hemoglobinuria as repeated acute kidney injury

Manish R Balwani 1 * , Vivek B Kute 1, Pankaj R Shah 1, Maulin Shah 1, Umesh Varyani 1, Hargovind L. Trivedi 1

1 Department of Nephrology and Clinical Transplantation, Institute of Kidney Diseases and Research Center, Dr. HL Trivedi Institute of Transplantation Sciences (IKDRC-ITS), Ahmedabad, India
*Corresponding author: Manish R Balwani, Email: balwani.manish@yahoo.com

Abstract

The triad of hemolytic anemia, pancytopenia, and thrombosis makes paroxysmal nocturnal hemoglobinuria (PNH) a truly unique clinical syndrome. Intravascular hemolysis in PNH can lead to a severe hemolytic episode with massive hemoglobinuria which can cause acute kidney injury (AKI) probably from acute tubular necrosis (ATN). A 15 -year-old girl was admitted with history of fever, diarrhea, vomiting followed by decreased urine output since 3 days. Urinalysis showed nil protein, no red blood cells (RBCs) on microscopy. Plasma hemoglobin level, total leukocyte count, platelet count, and serum creatinine were 6.5 gm/dl, 6440/μl, 205 000/μl, and 3.1 mg/dl, respectively. She received 3 units of packed red blood cells and the patient was discharged with normal renal function test with a diagnosis of acute gastroenteritis with recovered AKI. After 8 months she again had fever, vomiting, nausea with decreased urine output since 3 days. Laboratory investigations showed hemoglobin - 5.5 gm/dl, total leukocyte count - 1550/ μl, platelet count - 165000/μl, and serum creatinine - 4.89 mg/dl. Serum LDH level was 2188 U/l. She was managed conservatively with steroids, antibiotics and she recovered her kidney functions to normal in a week. Presentation of repeated AKI with hemolytic anemia in a short span after fever led us to perform flow cytometric analysis of peripheral blood granulocytes which revealed the presence of PNH clone. PNH may present with renal disease and anemia only even without classical history of hematuria or venous thrombosis. Thus it needs high index of suspicion as early diagnosis and treatment will help in preventing repeated episodes of AKI and thus chronic kidney disease.

Implication for health policy/practice/research/medical education:

Paroxysmal nocturnal hemoglobinuria (PNH) may present with renal disease and anemia only even without classical history of hematuria or venous thrombosis. Thus it needs early and high index of suspicion for PNH as early diagnosis and treatment will help in preventing repeated episodes of acute kidney injury (AKI) and thus chronic kidney disease (CKD).

Please cite this paper as: 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):116-118.

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First published online: 09 Sep 2015
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