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Dtsch Med Wochenschr 2025; 150(04): 167-172
DOI: 10.1055/a-2382-6433
Übersicht

Atypisches hämolytisch-urämisches Syndrom: Differentialdiagnostik und Therapie

Ein Diagnose- und Behandlungsleitfaden für die PraxisAtypical hemolytic uremic syndrome: differential diagnosis and therapyA clinical practice guideline for diagnosis and therapy
Anja Gäckler
,
Paul Thomas Brinkkötter
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Das atypische hämolytisch-urämische Syndrom ist eine wichtige Differenzialdiagnose bei thrombotischer Mikroangiopathie [1]. Da eindeutige Biomarker fehlen, handelt es sich um eine Diagnose im Ausschlussverfahren. Wenn eine adäquate Therapie ausbleibt oder verzögert erfolgt, ist die Prognose ungünstig. Umso wichtiger sind zeitnahe differenzialdiagnostische Abwägungen und Therapieeinleitung. Dieser Leitfaden soll hierbei eine Hilfestellung geben.

Abstract

Atypical hemolytic uremic syndrome (aHUS) is an important differential diagnosis in thrombotic microangiopathy (TMA). The absence of definitive biomarkers usually allows for aHUS to be diagnosed only through a process of exclusion. Due to the unfavorable prognosis if adequate therapy is delayed or not provided, differential diagnostic considerations and initiation of treatment must occur promptly. The presented guideline is intended to serve as an aid in this process.

Schlüsselwörter

thrombotische Mikroangiopathie - atypisches hämolytisch-urämisches Syndrom - Differentialdiagonse - Therapie

Keywords

thrombotic microangiopathy - atypical hemolytic uremic syndrome - differential diagnosis - therapy


Publikationsverlauf

Artikel online veröffentlicht:
29. Januar 2025

© 2025. Thieme. All rights reserved.

Georg Thieme Verlag KG
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  • Literatur

  • 1 Schönermarck U, Ries W, Schröppel B. et al. Relative incidence of thrombotic thrombocytopenic purpura and haemolytic uraemic syndrome in clinically suspected cases of thrombotic microangiopathy. Clin Kidney J 2019; 13 (02) 208-216
    CrossrefPubMedSuche in Google Scholar
  • 2 Yan K, Desai K, Gullapalli L. et al. Epidemiology of atypical hemolytic uremic syndrome: a systemic literature review. Clin Epidemiol 2020; 12: 295-305
    CrossrefPubMedSuche in Google Scholar
  • 3 Fakhouri F, Zuber J, Frémeaux-Bacchi V. et al. Haemolytic uraemic syndrome. Lancet 2017; 390: 681-696
    CrossrefPubMedSuche in Google Scholar
  • 4 Li A, Makar R, Hurwitz S. et al. Treatment with or without plasma exchange for patients with acquired thrombotic microangiopathy not associated with severe ADAMTS13 deficiency: a propensity score-matched study. Transfusion 2016; 56 (08) 2069-2077
    CrossrefPubMedSuche in Google Scholar
  • 5 Fremeaux-Bacchi V, Fakhouri F, Garnier A. et al. Genetics and outcome of atypical hemolytic uremic syndrome: a nationwide French series comparing children and adults. Clin J Am Soc Nephrol 2013; 8 (04) 554-562
    CrossrefPubMedSuche in Google Scholar
  • 6 Walle J, Delmas Y, Ardissino G. et al. Improved renal recovery in patients with atypical hemolytic uremic syndrome following rapid initiation of eculizumab treatment. J Nephrol 2017; 30 (01) 127-134
    CrossrefPubMedSuche in Google Scholar
  • 7 Jokiranta T, Viklicky O, Al Shorafa S. et al. Differential diagnosis of thrombotic microangiopathy in nephrology. BMC Nephrol 2017; 18 (01) 324
    CrossrefPubMedSuche in Google Scholar
  • 8 Bommer M, Wölfle-Guter M. et al. Differenzialdiagnose und Therapie thrombotischer Mikroangiopathien. Dtsch Arztebl Int 2018; 115 (19) 327-334
    CrossrefPubMedSuche in Google Scholar
  • 9 AWMF: S2k-Leitlinie Hämolytisch-Urämisches Syndrom im Kindesalter (Uptdate 2022). Zugriff am 23. August 2024 unter: https://register.awmf.org/de/leitlinien/detail/166–002
    PubMed
  • 10 Bendapudi P, Hurwitz S, Fry A. et al. Derivation and external validation of the PLASMIC score for rapid assessment of adults with thrombotic microangiopathies: a cohort study. Lancet Haematol 2017; 4 (04) e157-e164
    CrossrefPubMedSuche in Google Scholar
  • 11 Coppo P, Schwarzinger M, Buffet M. et al. Predictive features of severe acquired ADAMTS13 deficiency in idiopathic thrombotic microangiopathies: the French TMA reference center experience. PLoS One 2010; 5 (04) e10208
    CrossrefPubMedSuche in Google Scholar
  • 12 Fage N, Orvain C, Henry N. et al. Proteinuria increases the PLASMIC and French Scores performance to predict thrombotic thrombocytopenic purpura in patients with thrombotic microangiopathy syndrome. Kidney Int Rep 2021; 7 (02) 221-231
    CrossrefPubMedSuche in Google Scholar
  • 13 Legendre C, Licht C, Muus P. et al. Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome. N Engl J Med 2013; 368 (23) 2169-2181
    CrossrefPubMedSuche in Google Scholar
  • 14 Greenbaum L, Fila M, Ardissino G. et al. Eculizumab is a safe and effective treatment in pediatric patients with atypical hemolytic uremic syndrome. Kidney Int 2016; 89 (03) 701-711
    CrossrefPubMedSuche in Google Scholar
  • 15 Rondeau E, Scully M, Ariceta G. et al. The long-acting C5 inhibitor, ravulizumab, is effective and safe in adult patients with atypical hemolytic uremic syndrome naive to complement inhibitor treatment. Kidney Int 2020; 97 (06) 1287-1296
    CrossrefPubMedSuche in Google Scholar
  • 16 Tanaka K, Adams B, Aris A. et al. The long-acting C5 inhibitor, ravulizumab, is efficacious and safe in pediatric patients with atypical hemolytic uremic syndrome previously treated with eculizumab. Pediatr Nephrol 2021; 36 (04) 889-898
    CrossrefPubMedSuche in Google Scholar
  • 17 Haas C, Nitschke M, Menne J. et al. Charakterisierung von Patienten mit atypischem hämolytisch-urämischen Syndrom (aHUS) in Deutschland, Daten aus dem globalen aHUS-Register. Nephrologe 2019; 14: 496-504
    CrossrefPubMedSuche in Google Scholar