Unprecedented Haemorrhagic Stroke: A Rare Manifestation of Atypical Haemolytic Syndrome

Overview

Journal Cureus

Specialty Biomedical Engineering

Date 2024 Oct 28

PMID 39463586

Authors

Shahzaib Fida

Sucheta Sharma

Affiliations

Soon will be listed here.

Abstract

Atypical haemolytic uraemic syndrome (aHUS) is a rare and complex condition characterized by systemic thrombotic microangiopathy resulting from complement dysregulation. While primarily affecting renal microvasculature, aHUS can present with multi-organ involvement, posing significant diagnostic and therapeutic challenges. We report the case of a 22-year-old female with a history of aHUS who developed a catastrophic haemorrhagic stroke. Her clinical course underscores the severe and unpredictable nature of aHUS, illustrating the critical need for heightened awareness of its potential neurological manifestations. aHUS is typically triggered by a combination of genetic predisposition and environmental factors such as infections or medications. This case highlights the necessity for comprehensive evaluation and prompt intervention in patients with aHUS presenting with atypical symptoms. The complexity of aHUS necessitates a multidisciplinary approach to diagnosis and management to mitigate morbidity and mortality.

References

Sallee M, Daniel L, Piercecchi M, Jaubert D, Fremeaux-Bacchi V, Berland Y . Myocardial infarction is a complication of factor H-associated atypical HUS. Nephrol Dial Transplant. 2010; 25(6):2028-32. DOI: 10.1093/ndt/gfq160. View

Noris M, Caprioli J, Bresin E, Mossali C, Pianetti G, Gamba S . Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype. Clin J Am Soc Nephrol. 2010; 5(10):1844-59. PMC: 2974386. DOI: 10.2215/CJN.02210310. View

Vilalta R, Lara E, Madrid A, Chocron S, Munoz M, Casquero A . Long-term eculizumab improves clinical outcomes in atypical hemolytic uremic syndrome. Pediatr Nephrol. 2012; 27(12):2323-6. PMC: 3491201. DOI: 10.1007/s00467-012-2276-8. View

Besbas N, Gulhan B, Soylemezoglu O, Ozcakar Z, Korkmaz E, Hayran M . Turkish pediatric atypical hemolytic uremic syndrome registry: initial analysis of 146 patients. BMC Nephrol. 2017; 18(1):6. PMC: 5217238. DOI: 10.1186/s12882-016-0420-6. View

Sellier-Leclerc A, Fremeaux-Bacchi V, Dragon-Durey M, Macher M, Niaudet P, Guest G . Differential impact of complement mutations on clinical characteristics in atypical hemolytic uremic syndrome. J Am Soc Nephrol. 2007; 18(8):2392-400. DOI: 10.1681/ASN.2006080811. View

Mele C, Remuzzi G, Noris M . Hemolytic uremic syndrome. Semin Immunopathol. 2014; 36(4):399-420. DOI: 10.1007/s00281-014-0416-x. View

Noris M, Remuzzi G . Atypical hemolytic-uremic syndrome. N Engl J Med. 2009; 361(17):1676-87. DOI: 10.1056/NEJMra0902814. View

Malina M, Gulati A, Bagga A, Majid M, Simkova E, Schaefer F . Peripheral gangrene in children with atypical hemolytic uremic syndrome. Pediatrics. 2012; 131(1):e331-5. DOI: 10.1542/peds.2012-0903. View

Sawai T, Nangaku M, Ashida A, Fujimaru R, Hataya H, Hidaka Y . Diagnostic criteria for atypical hemolytic uremic syndrome proposed by the Joint Committee of the Japanese Society of Nephrology and the Japan Pediatric Society. Pediatr Int. 2014; 56(1):1-5. DOI: 10.1111/ped.12274. View

10.

Loirat C, Fakhouri F, Ariceta G, Besbas N, Bitzan M, Bjerre A . An international consensus approach to the management of atypical hemolytic uremic syndrome in children. Pediatr Nephrol. 2015; 31(1):15-39. DOI: 10.1007/s00467-015-3076-8. View

11.

Mallett A, Hughes P, Szer J, Tuckfield A, Van Eps C, Cambell S . Atypical haemolytic uraemic syndrome treated with the complement inhibitor eculizumab: the experience of the Australian compassionate access cohort. Intern Med J. 2015; 45(10):1054-65. DOI: 10.1111/imj.12864. View

12.

Dunn S, Brocklebank V, Bryant A, Carnell S, Chadwick T, Johnson S . Safety and impact of eculizumab withdrawal in patients with atypical haemolytic uraemic syndrome: protocol for a multicentre, open-label, prospective, single-arm study. BMJ Open. 2022; 12(9):e054536. PMC: 9486193. DOI: 10.1136/bmjopen-2021-054536. View

13.

Noris M, Remuzzi G . Cardiovascular complications in atypical haemolytic uraemic syndrome. Nat Rev Nephrol. 2014; 10(3):174-80. DOI: 10.1038/nrneph.2013.280. View

14.

Loirat C, Fremeaux-Bacchi V . Atypical hemolytic uremic syndrome. Orphanet J Rare Dis. 2011; 6:60. PMC: 3198674. DOI: 10.1186/1750-1172-6-60. View

15.

Nurnberger J, Philipp T, Witzke O, Opazo Saez A, Vester U, Baba H . Eculizumab for atypical hemolytic-uremic syndrome. N Engl J Med. 2009; 360(5):542-4. DOI: 10.1056/NEJMc0808527. View

16.

Salem G, Flynn J, Cataland S . Profound neurological injury in a patient with atypical hemolytic uremic syndrome. Ann Hematol. 2012; 92(4):557-8. DOI: 10.1007/s00277-012-1615-y. View

17.

Johnson S, Stojanovic J, Ariceta G, Bitzan M, Besbas N, Frieling M . An audit analysis of a guideline for the investigation and initial therapy of diarrhea negative (atypical) hemolytic uremic syndrome. Pediatr Nephrol. 2014; 29(10):1967-78. DOI: 10.1007/s00467-014-2817-4. View

18.

Roman-Ortiz E, Mendizabal Oteiza S, Pinto S, Lopez-Trascasa M, Sanchez-Corral P, Rodriguez de Cordoba S . Eculizumab long-term therapy for pediatric renal transplant in aHUS with CFH/CFHR1 hybrid gene. Pediatr Nephrol. 2013; 29(1):149-53. DOI: 10.1007/s00467-013-2591-8. View

19.

Campistol J, Arias M, Ariceta G, Blasco M, Espinosa L, Espinosa M . An update for atypical haemolytic uraemic syndrome: diagnosis and treatment. A consensus document. Nefrologia. 2015; 35(5):421-47. DOI: 10.1016/j.nefro.2015.07.005. View

20.

Ozel A, Caliskan U, Gucer S . Peripheral gangrene complicating hemolytic uremic syndrome in a child. Pediatr Nephrol. 2003; 18(5):465-7. DOI: 10.1007/s00467-002-0973-4. View