Zeynep YÜRÜK YILDIRIM, Alev YILMAZ, Sevinç EMRE, Ilmay BİLGE, Bağdagül YAVAŞ AKSU, Aydan ŞİRİN

Kompleman Faktör H Antikoru İlişkili Atipik Hemolitik Üremik Sendrom ve Ekulizumab Tedavisi

Atipik hemolitik üremik sendrom aHÜS , sıklıkla son dönem böbrek yetersizliğine ilerleyen ender görülen kro- nik bir hastalıktır. Bu hastaların çoğunda alternatif komp- leman yolağının bozulmasına neden olan genetik veya edinsel bozukluk vardır. Kompleman Faktör H otoantikor- ları aHÜS’lü vakaların %6-11’inde bildirilmiştir. Plazma değişimi ve immunsupresif tedaviye rağmen, relapsları önlenemeyen, ancak Ekulizumab kullanımı ile remisyon sağlanan bir anti-FH antikor ilişkili aHÜS vakasını sun- mayı amaçladık

Anahtar Kelimeler:

Atipik hemolitik üremik sendrom, kompleman faktör H antikorları, Ekulizumab tedavisi

Complement Factor H Antibody-Associated Atypical Hemolytic Uremic Syndrome and Eculizumab Treatment

Atypical hemolytic uremic syndrome aHUS is a rare, chronic disease with frequent progression to end-stage renal disease. In the majority of these patients, there is a genetic or acquired disorder that causes the dysregulation of the alternative complement pathway. Factor H autobodi- es have been reported in 6-11% of the patients with aHUS. We aimed to present a case with anti-FH antibody- associated aHUS with intractable relapses despite plasma exchange and immunosuppressive treatment, but remission was provided by the use of eculizumab

Keywords:

Atypical hemolytic uremic syndrome, factor H autoantibodies, eculizumab therapy,

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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;85:905-909.
Ariceta g, Besbas n, Johnson s, Karpman D, landau D, licht C, et al. Guideline for the investiga- tion and initial therapy of diarrhea-negative hemolytic uremic syndrome. Pediatr Nephrol 2009;24:687-96.
https://doi.org/10.1007/s00467-008-0964-1
Dragon-Durey MA, loirat C, Cloarec s, Macher MA, Blouin J, nivet H, et al. Anti-Factor H auto anti- bodies associated with atypical hemolytic uremic syndrome. J Am Soc Nephrol 2005;16:555-63.
https://doi.org/10.1681/ASN.2004050380
Józsi M, strobel s, Dahse HM, liu Ws, Hoyer PF, Oppermann M, et al. Anti factor H autoantibodies block C-terminal recognition function of factor H in hemolytic uremic syndrome. Blood 2007;110:1516-8.
https://doi.org/10.1182/blood-2007-02-071472
Józsi M, licht C, strobel s, Zipfel sl, richter H, Heinen s, et al. Factor H autoantibodies in atypical hemolytic uremic syndrome correlate with CFHR1/ CFHR3 deficiency. Blood 2008;111:1512-4.
https://doi.org/10.1182/blood-2007-09-109876
Waters AM, licht C. aHUS caused by complement dysregulation: New therapies on the horizon. Pediatr Nephrol 2011;26:41-57.
https://doi.org/10.1007/s00467-010-1556-4
Hillmen P, Hall C, Marsh JC, elebute M, Bombara MP, Petro Be, et al. Effect of eculizumab on hemoly- sis and transfusion requirements in patients with paroxysmal nocturnal hemoglobinuria. N Engl J Med 2004;350:552-9.
https://doi.org/10.1056/NEJMoa031688
loirat C, Fakhouri F, Ariceta g, Besbas n, Bitzan M, Bjerre A, et al. An international consensus appro- ach to the management of atypical hemolytic uremic syndrome in children. Pediatric Nephrol 2016;31(1):15- 39.
https://doi.org/10.1007/s00467-015-3076-8
vaisbich MH. Hemolytic-uremic syndrome in child- hood. J Bras Nefrol 2014;36:208-20.
https://doi.org/10.5935/0101-2800.20140032
sinha A, gulati A, saini s, Blanc C, gupta A, gurjar Bs, et al. Prompt plasma exchanges and immunosuppressive treatment improves the outcomes of anti-factor H autoantibody- associated hemolytic uremic syndrome in children. Kidney Int 2014;85:1151- 60.
https://doi.org/10.1038/ki.2013.373
salvadori M, Bertoni e. Update on hemolytic uremic syndrome: Diagnostic and therapeutic recommendati- ons. World J Nephrol 2013;2:56-76.
https://doi.org/10.5527/wjn.v2.i3.56
Diamante Chiodini B, Davin JC, Corazza F, Khaldi K, Dahan K, Ismaili K, et al. Eculizumab in anti- factor h antibodies associated with atypical hemolytic uremic syndrome. Pediatrics 2014;133:e1764-e1768.
https://doi.org/10.1542/peds.2013-1594
green H, Harari e, Davidovits M, Blickstein D, grossman A, gafter u, et al. Atypical HUS due to factor H antibodies in an adult patient successfully treated with eculizumab. Ren Fail 2014;36:1119-21.
https://doi.org/10.3109/0886022X.2014.917574