A Case of Childhood Severe Paroxysmal Cold Hemoglobinuria with Acute Renal Failure Successfully Treated with Plasma Exchange and Eculizumab

Abstract

We describe the case of a 4-year-old female who presented with sepsis and disseminated intravascular coagulation (DIC), developed ongoing intravascular hemolysis with acute renal failure from suspected pigment-induced acute tubular necrosis necessitating continuous renal replacement therapy (CRRT) for five days followed by four episodes of intermittent hemodialysis (iHD), and was subsequently diagnosed with paroxysmal cold hemoglobinuria (PCH). She was successfully treated with plasma exchange and eculizumab, a humanized monoclonal antibody targeting complement protein C5, and demonstrated significant improvement of hemolysis and recovery of renal function.

Severe Acute Kidney Injury in Children as a Rare Complication of Paroxysmal Cold Hemoglobinuria

“…There are also reports of plasmapheresis therapy trials in severe cases with the aim of removing D-L antibody. To date, only individual cases documenting improvement of AKI (2 pediatric and 1 adult case) were reported 7,15,16 In our small case series only 3 of 26 AIHA patients required RRT. Severe AKI was treated by CVVHD and transient intermittent HD (case 3) and resolved within few weeks in all cases.…”

A single center experience for clinical evaluation of paroxysmal cold hemoglobinuria and Donath‐Landsteiner testing

“…We did observe a more rapid increase in Hgb acutely in our pediatric cases, but this was not significant. In extraordinary cases, the use of eculizumab and plasma exchange has been shown to be beneficial, but these interventions were not used in any of our pediatric or adult patients 5,14 . Our findings were least clear in the adult population, as both DL‐positive patients received multiple interventions alongside steroids, which are also treatments for their underlying disease states driving hemolysis.…”

“…In extraordinary cases, the use of eculizumab and plasma exchange has been shown to be beneficial, but these interventions were not used in any of our pediatric or adult patients. 5,14 Our findings were least clear in the adult population, as both DL-positive patients received multiple interventions alongside steroids, which are also treatments for their underlying disease states driving hemolysis. Future studies with larger pediatric cohorts are needed to determine if a short course of steroids has any benefits in a subset of moderately to severely anemic patients versus just supportive care.…”

“…Based on the literature, initial administration of steroids is common for PCH patients on presentation. 7,14,15 It's been suggested that the impact of steroids in PCH is unclear, given the transient nature of the disease. 2 In our study cohort, we observed that the administration of steroids had no impact on either shortor long-term recovery, as most pediatric patients had nearnormal hemoglobin levels at 4 weeks.…”

“…In our study cohort, we observed that only 33% of the DL‐positive pediatric patients received steroids upon presentation when compared to the 90% of the pediatric DL‐negative patients. Based on the literature, initial administration of steroids is common for PCH patients on presentation 7,14,15 . It's been suggested that the impact of steroids in PCH is unclear, given the transient nature of the disease 2 .…”