Repeated spontaneous remission of atypical hemolytic-uremic syndrome caused by influenza – a case report

Czech version

Authors: H. Flögelová ¹; J. Volejníková ¹; I. Hrachovinová ²; Z. Prohászka ³; M. Šeda ¹; J. Gumulec ⁴
Authors‘ workplace: Dětská klinika LF Univerzity Palackého a Fakultní nemocnice Olomouc, Česká republika ¹; Ústav hematologie a krevní transfuze Praha (ÚHKT), Česká republika ²; Research Laboratory, Semmelweis University, 3rd Department of Internal Medicine, Budapest, Hungary ³; Klinika hematoonkologie, Fakultní nemocnice Ostrava, Česká republika ⁴
Published in: Čes-slov Pediat 2020; 75 (2): 98-102.
Category: Case Report

Overview

Here we report spontaneous recovery of 2 episodes of atypical hemolytic-uremic syndrome (aHUS) in a 17-year-old male. In both cases, the condition was caused by seasonal influenza (first, influenza A/H3; second, after 1 year, influenza B). Already at the first onset of aHUS, decreased expression of CD46 (membrane cofactor protein, MCP) on granulocytes was found, suggesting the diagnosis of complement-mediated HUS. Because the patient’s clinical condition was stable and laboratory results were improving since day 3, we decided not to administer eculizumab, a drug whose administration is recommended within the first 48 hours in children, but to continue symptomatic therapy only. However, eculizumab was available in case that the patient’s clinical condition deteriorated. Spontaneous remission of HUS was achieved in both episodes of aHUS. Considering the presumed risk of aHUS relapse, genetic examinations were done following the first episode. The patient was confirmed to carry a mutation in MCP (CD46) gene (heterozygote for the p.C35Y mutation) and also a high-risk MCPggaac haplotype of the same gene. We assume that both mentioned genetic lesions played a pathogenic role in the development of complement-mediated aHUS, with influenza being the triggering condition. Molecular genetic tests were also performed in the patient’s parents and found accordingly, that the father is a heterozygote for the p.C35Y mutation and the mother is heterozygous for the high-risk MCPggaac haplotype of the MCP (CD46) gene.

We confirmed that in an individual with a mutation in MCP, seasonal influenza may be a trigger of aHUS and spontaneous remission is possible.

Keywords:

remission – influenza – atypical hemolytic-uremic syndrome (HUS) – membrane cofactor protein (MCP)

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