Erişkinlerde Hemolitik Üremik Sendrom (HÜS)
Özet
Hemolitik Üremik Sendromu (HÜS), mikroanjiyopatik hemolitik anemi, trombositopeni ve akut böbrek hasarının birlikteliği ile karakterize, trombotik mikroanjiyopatiler (TMA) spektrumunda yer alan bir klinik tablodur. Erişkinlerde görülme sıklığı çocukluk çağına göre daha düşüktür; ancak fenotipik çeşitlilik, eşlik eden komorbiditeler ve sekonder tetikleyicilerin fazlalığı tanı ve tedavide güçlük oluşturur. Bu bölümde, doğumdan kısa süre sonra hastaneye başvuran; ardışık plazmaferez ile stabilize edilip eculizumabla idame edilen 26 yaşındaki bir olgu üzerinden epidemiyoloji, etiyoloji, patofizyoloji, klinik özellikler, ayırıcı tanı, tedavi ve izlem başlıkları bütüncül biçimde ele alınmıştır.
Referanslar
Gasser C, Gautier E, Steck A, Siebenmann RE, Oechslin R. [Hemolytic-uremic syndrome: bilateral necrosis of the renal cortex in acute acquired hemolytic anemia]. Schweiz Med Wochenschr. 1955;85(38-39):905-9.
Zhang K, Lu Y, Harley KT, Tran MH. Atypical Hemolytic Uremic Syndrome: A Brief Review. Hematol Rep. 2017;9(2):7053.
Mayer CL, Leibowitz CS, Kurosawa S, Stearns-Kurosawa DJ. Shiga toxins and the pathophysiology of hemolytic uremic syndrome in humans and animals. Toxins (Basel). 2012;4(11):1261-87.
Kavanagh D, Raman S, Sheerin NS. Management of hemolytic uremic syndrome. F1000Prime Reports. 2014;6:119.
Doshi K, Yusuf A, Licht C, Boyer O, Nester C, Murra A, et al. Extrarenal manifestations of atypical hemolytic uremic syndrome: a systematic review and meta-analysis. Pediatr Res. 2024.
Frank C, Werber D, Cramer JP, Askar M, Faber M, an der Heiden M, et al. Epidemic profile of Shiga-toxin-producing Escherichia coli O104:H4 outbreak in Germany. N Engl J Med. 2011;365(19):1771-80.
Salvadori M, Bertoni E. Update on hemolytic uremic syndrome: Diagnostic and therapeutic recommendations. World J Nephrol. 2013;2(3):56-76.
Yan K, Desai K, Gullapalli L, Druyts E, Balijepalli C. Epidemiology of Atypical Hemolytic Uremic Syndrome: A Systematic Literature Review. Clin Epidemiol. 2020;12:295-305.
Gupta M, Govindappagari S, Burwick RM. Pregnancy-Associated Atypical Hemolytic Uremic Syndrome: A Systematic Review. Obstet Gynecol. 2020;135(1):46-58.
Le Clech A, Simon-Tillaux N, Provôt F, Delmas Y, Vieira-Martins P, Limou S, et al. Atypical and secondary hemolytic uremic syndromes have a distinct presentation and no common genetic risk factors. Kidney Int. 2019;95(6):1443-52.
Brady TM, Pruette C, Loeffler LF, Weidemann D, Strouse JJ, Gavriilaki E, et al. Typical Hus: Evidence of Acute Phase Complement Activation from a Daycare Outbreak. J Clin Exp Nephrol. 2016;1(2).
Bhandari J, Rout P, Sedhai YR. Hemolytic uremic syndrome. StatPearls [Internet]: StatPearls Publishing; 2023.
Feng S, Eyler SJ, Zhang Y, Maga T, Nester CM, Kroll MH, et al. Partial ADAMTS13 deficiency in atypical hemolytic uremic syndrome. Blood. 2013;122(8):1487-93.
Tsai HM, Chandler WL, Sarode R, Hoffman R, Jelacic S, Habeeb RL, et al. von Willebrand factor and von Willebrand factor-cleaving metalloprotease activity in Escherichia coli O157:H7-associated hemolytic uremic syndrome. Pediatr Res. 2001;49(5):653-9.
Lee MS, Cherla RP, Tesh VL. Shiga toxins: intracellular trafficking to the ER leading to activation of host cell stress responses. Toxins (Basel). 2010;2(6):1515-35.
Freedman SB, van de Kar N, Tarr PI. Shiga Toxin-Producing Escherichia coli and the Hemolytic-Uremic Syndrome. N Engl J Med. 2023;389(15):1402-14.
Blasco M, Guillén-Olmos E, Diaz-Ricart M, Palomo M. Complement Mediated Endothelial Damage in Thrombotic Microangiopathies. Front Med (Lausanne). 2022;9:811504.
Tonkovic U, Bogicevic M, Manzar A, Andrejic N, Sic A, Atanaskovic M, et al. Neurological Manifestations of Hemolytic Uremic Syndrome: A Comprehensive Review. Brain Sci. 2025;15(7).
Khalid M, Andreoli S. Extrarenal manifestations of the hemolytic uremic syndrome associated with Shiga toxin-producing Escherichia coli (STEC HUS). Pediatr Nephrol. 2019;34(12):2495-507.
Thomas K, Ananthula A, Lopez-Flores R, Toro AD, Chapple AG, Loch M. The Use of Eculizumab for the Treatment of Atypical Hemolytic Uremic Syndrome in an Academic Hematology Center. Perm J. 2023;27(2):23-30.
Dixon BP, Kavanagh D, Aris ADM, Adams B, Kang HG, Wang E, et al. Ravulizumab in Atypical Hemolytic Uremic Syndrome: An Analysis of 2-Year Efficacy and Safety Outcomes in 2 Phase 3 Trials. Kidney Med. 2024;6(8):100855.
Praga M, Rodríguez de Córdoba S. Secondary atypical hemolytic uremic syndromes in the era of complement blockade. Kidney International. 2019;95(6):1298-300.
Hans R, Sharma RR, Marwaha N, Suri D, Kumar R, Gupta A, et al. Efficacy and safety of therapeutic plasma exchange by using apheresis devices in pediatric atypical hemolytic uremic syndrome patients. J Clin Apher. 2016;31(4):381-7.
