Thrombotic microangiopathy discribes a specific pathologic lesion in which abnormalities in the vessel wall of arterioles and capillaries lead to microvascular thrombosis. Microangiopathic hemolytic anemia is a descriptive term for non-immun hemolytic anemia resulting from intravascular red blood cell fragmentation that produces schistocytes on the peripheral blood smear. Atypical hemolytic uremic syndrome is a type of primer thrombotic microangiopathy and using eculizumab in the early phase of therapy reduces the risk of irreversible renal damage. We mentioned both the importance of early diagnosis at the patients presenting with microangiopathic hemolytic anemia and thrombocytopenia like our patient and the benefit of eculizumab therapy at the early phase of atypical hemolytic uremic syndrome therapy.
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.
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