Tugay AVCI, İbrahim VASİ, İlter BOZACI, Gökhan TEMİZ

Atipik Hemolitik Üremik Sendrom Olgusunda Eculizumab Deneyimi

Trombotik mikroanjiyopati, arteriollerin ve kapiller damarların duvarlarındaki mikrovasküler tromboz gelişimine neden olan anormallikleri tanımlayan spesifik bir patolojik lezyondur. Mikroanjiyopatik hemolitik anemi ise, periferik yaymada şistosit oluşumuna neden olan, damar içindeki kırmızı kan hücrelerinin fragmantasyonundan kaynaklanan non-immün bir hemolitik anemi tipidir. Atipik hemolitik üremik sendrom (AHÜS), primer trombotik mikroanjiyopati nedenlerinden biri olup tedavide erken dönemde eculizumab kullanımı geri dönüşümsüz böbrek hasarı riskini azaltmaktadır. Mikroanjiyopatik hemolitik anemi ve trombositopeni ile gelen olgumuzda hem erken tanısal yaklaşımın önemini hem de atipik hemolitik üremik sendrom (AHÜS) olgularında erken eculizumab kullanımının böbrek fonksiyonları üzerine olan olumlu etkisini vurgulamayı amaçladık.

Eculizumab Experince at a Patient With Atypical Hemolitic Uremic Syndrome

Thrombotic microangiopathy discribes a specific pathologiclesion in which abnormalities in the vessel wall of arteriolesand capillaries lead to microvascular thrombosis.Microangiopathic hemolytic anemia is a descriptive term fornon-immun hemolytic anemia resulting from intravascular redblood cell fragmentation that produces schistocytes on theperipheral blood smear. Atypical hemolytic uremic syndromeis a type of primer thrombotic microangiopathy and usingeculizumab in the early phase of therapy reduces the risk ofirreversible renal damage. We mentioned both the importanceof early diagnosis at the patients presenting withmicroangiopathic hemolytic anemia and thrombocytopenialike our patient and the benefit of eculizumab therapy at theearly phase of atypical hemolytic uremic syndrome therapy.

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Laszik ZG, Kambham N, Silva FG. Thrombotic microangiopathies. In: Heptinstall's Pathology of the Kidney, Jennett JC, D'Agati VD, Olson JL, et al. (Eds), Lippincott Williams & Wilkins, Philadelphia 2014.
George JN, Nester CM. Syndromes of thrombotic microangiopathy. N Engl J Med 2014; 371:654-656.
Brain MC, Dacie JV, Hourihane DO. Microangiopathic haemolytic anaemia: the possible role of vascular lesions in pathogenesis. Br J Haematol 1962; 8:358-362.
Noris M, Caprioli J, Bresin E, Mossali C, Pianetti G, et al. Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype. Clin J Am Soc Nephrol 2010; 5:1844-1848.
Sellier-Leclerc AL, Fremeaux-Bacchi V, Dragon-Durey MA, et al. Differential impact of complement mutations on clinical characteristics in atypical hemolytic uremic syndrome. J Am Soc Nephrol 2007; 18:2392-2393.
Al-Nouri ZL, Reese JA, Terrell DR, Vesely SK,George JN. Drug-induced thrombotic microangiopathy: a systematic review of published reports. Blood 2015; 125:616-618.
George JN. Cobalamin C deficiency-associated thrombotic microangiopathy: uncommon or unrecognised. Lancet 2015; 386:1012-1013
Booth KK, Terrell DR, Vesely SK, George JN. Systemic infections mimicking thrombotic thrombocytopenic purpura. Am J Hematol 2011; 86:743-745.
George JN. Systemic malignancies as a cause of unexpected microangiopathic hemolytic anemia and thrombocytopenia. Oncology (Williston Park) 2011; 25:908-910.
Song D, Wu LH, Wang FM, Yang XW,Zhu D,Chen M, et al. The spectrum of renal thrombotic microangiopathy in lupus nephritis. Arthritis Res Ther 2013; 15:R12.