A Case of Atypical Hemolytic Uremic Syndrome Successfully Treated with Eculizumab (original) (raw)

Abstract

sparkles

AI

Idiopathic atypical hemolytic uremic syndrome (aHUS) is a rare and severe thrombotic microangiopathy characterized by hemolytic anemia, thrombocytopenia, and acute renal failure due to uncontrolled terminal complement activation. Patients often experience high morbidity and mortality rates despite supportive care. This report presents a case of a 51-year-old man with aHUS successfully treated with eculizumab, demonstrating its effectiveness in reversing hematologic abnormalities and preventing the need for dialysis.

Loading...

Loading Preview

Sorry, preview is currently unavailable. You can download the paper by clicking the button above.

References (11)

  1. Noris M, Remuzzi G: Atypical hemolytic-uremic syndrome. N Engl J Med 2009;361:1676-1687.
  2. Caprioli J, Noris M, Brioschi S, Pianetti G, Castelletti F, Bettinaglio P, Mele C, Bresin E, Cassis L, Gamba S, Porrati F, Bucchioni S, Monteferrante G, Fang CJ, Liszewski MK, Kavanagh D, Atkinson JP, Remuzzi G: Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome. Blood 2006;108:1267-1279.
  3. Noris M, Caprioli J, Bresin E, Mossali C, Pianetti G, Gamba S, Daina E, Fenili C, Castelletti F, Sorosina A, Piras R, Donadelli R, Maranta R, van der Meer I, Conway EM, Zipfel PF, Goodship TH, Remuzzi G: Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype. Clin J Am Soc Nephrol 2010;5:1844-1859.
  4. Hodgkins KS, Bobrowski AE, Lane JC, Langman CB: Clinical grand rounds: atypical hemolytic uremic syndrome. Am J Nephrol 2012;35:394-400.
  5. Loirat C, Frémeaux-Bacchi V: Atypical hemolytic uremic syndrome. Orphanet J Rare Dis 2011;6:60.
  6. Mache CJ, Acham-Roschitz B, Frémeaux-Bacchi V, Kirschfink M, Zipfel PF, Roedl S, Vester U, Ring E: Complement inhibitor eculizumab in atypical hemolytic uremic syndrome. Clin J Am Soc Nephrol 2009;4:1312-1316.
  7. Krisinger MJ, Goebeler V, Lu Z, Meixner SC, Myles T, Pryzdial EL, Conway EM: Thrombin generates previously unidentified C5 products that support the terminal complement activation pathway. Blood 2012;120:1717-1725.
  8. Amara U, Rittirsch D, Flierl M, Bruckner U, Klos A, Gebhard F, Lambris JD, Huber-Lang M: Interaction between the coagulation and complement system. Adv Exp Med Biol 2008;632:71-79.
  9. 9 Ohanian M, Cable C, Halka K: Eculizumab safely reverses neurologic impairment and eliminates need for dialysis in severe atypical hemolytic uremic syndrome. Clin Pharmacol 2011;3:5-12.
  10. Licht C, Muus P, Legendre C, Douglas K, Hourmant M, Delmas Y, Herthelius M, Delmas Y, Herthelius M, Trivelli A, Goodship T, Bedrosian CL, Loirat C: Long-term eculizumab therapy is effective in adolescent/adult patients with atypical hemolytic-uremic syndrome (aHUS) previously treated with chronic plasma exchange/infusion (PE/PI). Am Soc Nephrol, Kidney Week, Philadelphia, November 2011.
  11. Licht C, Muus P, Legendre C, Douglas K, Hourmant M, Delmas Y, Herthelius B, Trivelli A, Goodship T, Bedrosian CL, Loirat C: Eculizumab is an effective long-term treatment in patients with atypical hemolytic- uremic syndrome (aHUS) previously receiving chronic plasma exchange/infusion (PE/PI): extension study results. Am Soc Hematol, San Diego, December 2011.