İnönü Üniversitesi Turgut Özal Tıp Merkezi hematoloji kliniğine trombotik mikroanjiopati ile başvuran hastaların etyolojisinin retrospektif değerlendirilmesi ve plazma değişiminin tedavideki rolü
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2018
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İnönü Üniversitesi
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GİRİŞ Trombotik Mikroanjiopati (TMA); mikroanjiopatik hemolitik anemi (MAHA), trombositopeni ve mikrotrombüslerin yol açtığı iskemik doku hasarı ile karakterize bir hastalık grubudur. TMA'daki mikrotrombüsler trombosit agregatlarından meydana gelir. Periferik yaymada şiştosit varlığı, mekanik hemolize bağlı laboratuar bulguları (non-immün hemolitik anemi, laktat dehidrojenaz artışı, indirek bilirubin artışı, haptoglobulin azalması ve retikülositoz) ve rutin koagülasyon parametrelerinin normal olması TMA tanısını destekler. TMA primer ve sekonder olmak üzere iki gruba ayrılır. Primer grupta; Trombotik trombositopenik purpura (TTP) ve Hemolitik Üremik Sendrom (HÜS), sekonder grupta ise; gebelik, otoimün hastalıklar, malignite, kemik iliği transplantasyonu ve bazı ilaçların kullanımı yer almaktadır. Plazmaferez, hastanın plazmasının ayrıştırılıp yerine taze donmuş plazma, albümin, kriopresipitat, hidroksietilstarch gibi replasman sıvılarının konulması işlemidir. Terapötik plazma değişimi (TPD) ise replasman sıvısı olarak allojenik plazmanın kullanıldığı, plazmanın tedavi amaçlı değişimi işlemidir. Çalışmamız da hastanemize trombositopeni ve MAHA ile başvurup TMA tanısı alan hasta grubunda etyolojik sınıflandırmayı yapmayı ve bu hastaların hepsine uyguladığımız TPD'nin laboratuar parametrelerinde iyileşme ve mortalite üzerindeki etkisini değerlendirmeyi amaçladık. METOD Çalışmamızda 2009-2017 tarihleri arasında İnönü Üniversitesi Tıp Fakültesi Hematoloji Kliniğinde laboratuar ve periferik yayma değerlendirmesi ile TMA tanısı konulan 85 hasta retrospektif olarak incelendi. Hastalara iki yollu diyaliz katateri takılarak TPD tedavisi uygulandı. TPD tedavisi öncesi ve sonrası tam kan sayımı ve biyokimya parametreleri incelendi. BULGULAR TMA etyolojik sınıflandırmasında bölgemizde en sık HELLP sendromu, ikinci TTP, üçüncü yılan sokması ve en az oranda da atipik HÜS'e rastlandı. Hastaların hepsine TPD tedavisi uygulandı. Bu dört etyolojik sınıf içinde HELLP sendromu, TTP ve yılan sokmasında tedavide TPD uygulaması, prognoz üzerinde etkili laboratuvar parametrelerinde istatistiksel olarak anlamlı bir düzelme sağlamıştır (p<0.05). Atipik HÜS'te ise TPD'nin laboratuvar parametreleri üzerine etkinliğine bakıldığında istatistiksel olarak anlamlı düzelme izlenmedi (p>0.05). TPD uygulanan dört hastalık grubunun mortalite oranları incelendiğinde HELLP Sendromunda %9.7, TTP'de %21.7, atipik HÜS'te %27.3 ve yılan sokması %0 olarak bulundu. TARTIŞMA TMA etyolojik nedenleri bölgesel farklılıklar göstermektedir. Bölgemizde TMA grubu hastalar sık oranda bulunup, etyolojide özellikle en sık HELLP sendromu olmakla birlikte TTP ve yılan sokmasına da sık olarak rastlanmaktadır. Trombositopeni ve MAHA ile başvuran hastalarda TMA akla gelmeli ve etyolojisi en kısa sürede tespit edilmelidir. Ancak TMA etyolojisinin kesinleşmesini beklemeden mortalite ve morbidite üzerinde olumlu etkisi olması nedeniyle bir an önce TPD tedavisi başlanmalıdır. Anahtar kelimeler: Trombotik mikroanjiopati, Terapötik plazma değişimi, TTP, HELLP sendromu, Yılan Sokması, Atipik Hemolitik Üremik Sendrom
INTRODUCTION Thrombotic Mcroangiopathy (TMA); microangiopathic hemolytic anemia (MAHA) is a disease group characterized by ischemic tissue damage caused by thrombocytopenia and microthrombi. The microthromboses in TMA come from platelet aggregates. Presence of schistocytes in peripheral blood smear, laboratory hematological anemia (non-immunohemolytic anemia, increased lactate dehydrogenase, indirect bilirubin increase, decreased haptoglobulin and reticulocytosis) and normal routine coagulation parameters support TMA diagnosis. TMA is divided into two groups, primer and secondary. In the primer group; Thrombotic thrombocytopenic purpura (TTP) and Hemolytic Uremic Syndrome (HUS) were observed in the second group; pregnancy, autoimmune diseases, malignancy, bone marrow transplantation and the use of certain medicines. Plasmapheresis is the process of separating the patient's plasma and replacing it with replacement liquids such as fresh frozen plasma, albumin, cryoprecipitate, hydroxyethylstarch. Therapeutic plasma exchange (TPD) is the process of therapeutic substitution of the plasmid in which allogeneic plasma is used as the replacement fluid. In our study, we aimed to make an etiological classification in the patient group who received TMA diagnosis with thrombocytopenia and MAHA in our hospital and evaluate TPD's effect on the improvement of laboratory parameters and mortality. METHOD In our study, between 2009-2017, in Inonu University Medicine Faculty Hematology Clinic, 85 patients with TMA diagnosed with laboratory and peripheral blood smear examined retrospectively. TPD treatment was performed by attaching a two-way dialysis catheter to the patient. Complete blood count and biochemical parameters were examined before and after TPD treatment. BULGULAR In the etiological classification of TMA, in our region, HELLP syndrome was found the most frequently, second TTP, third snake bite and atypical HUS. TPD treatment was applied to all patients. In these four etiological classes, TPD treatment in HELLP syndrome, TTP and snake bite is effective on prognosis and statistically significant improvement in laboratory parameters (p<0.05). In the case of atypical HUS, no statistically significant improvement was observed when the efficacy of TPD on laboratory parameters (p>0.05). When the mortality rates of the four disease groups treated with TPD were examined, it was 9.7% in HELLP Syndrome, 21.7% in TTP, 27.3% in atypical HUS and 0% in snake bite. DISCUSSION Etiologic causes of TMA show regional differences. In our region, TMA group patients are frequently found and although it is espacially seen the most HELLP syndrome in etiology, TTP and snake bite are also common. In patients presenting with thrombocytopenia and MHA, TMA should be suspected and the etiology should be established as soon as possible. However, TPD treatment should be started as soon as possible because of the positive effect on mortality and morbidity without waiting for the definitive TMA etiology. Key words: Thrombotic microangiopathy, Therapeutic plasma exchange, TTP, HELLP syndrome, Snake bite, Atypical Hemolytic Uremic Syndrome
INTRODUCTION Thrombotic Mcroangiopathy (TMA); microangiopathic hemolytic anemia (MAHA) is a disease group characterized by ischemic tissue damage caused by thrombocytopenia and microthrombi. The microthromboses in TMA come from platelet aggregates. Presence of schistocytes in peripheral blood smear, laboratory hematological anemia (non-immunohemolytic anemia, increased lactate dehydrogenase, indirect bilirubin increase, decreased haptoglobulin and reticulocytosis) and normal routine coagulation parameters support TMA diagnosis. TMA is divided into two groups, primer and secondary. In the primer group; Thrombotic thrombocytopenic purpura (TTP) and Hemolytic Uremic Syndrome (HUS) were observed in the second group; pregnancy, autoimmune diseases, malignancy, bone marrow transplantation and the use of certain medicines. Plasmapheresis is the process of separating the patient's plasma and replacing it with replacement liquids such as fresh frozen plasma, albumin, cryoprecipitate, hydroxyethylstarch. Therapeutic plasma exchange (TPD) is the process of therapeutic substitution of the plasmid in which allogeneic plasma is used as the replacement fluid. In our study, we aimed to make an etiological classification in the patient group who received TMA diagnosis with thrombocytopenia and MAHA in our hospital and evaluate TPD's effect on the improvement of laboratory parameters and mortality. METHOD In our study, between 2009-2017, in Inonu University Medicine Faculty Hematology Clinic, 85 patients with TMA diagnosed with laboratory and peripheral blood smear examined retrospectively. TPD treatment was performed by attaching a two-way dialysis catheter to the patient. Complete blood count and biochemical parameters were examined before and after TPD treatment. BULGULAR In the etiological classification of TMA, in our region, HELLP syndrome was found the most frequently, second TTP, third snake bite and atypical HUS. TPD treatment was applied to all patients. In these four etiological classes, TPD treatment in HELLP syndrome, TTP and snake bite is effective on prognosis and statistically significant improvement in laboratory parameters (p<0.05). In the case of atypical HUS, no statistically significant improvement was observed when the efficacy of TPD on laboratory parameters (p>0.05). When the mortality rates of the four disease groups treated with TPD were examined, it was 9.7% in HELLP Syndrome, 21.7% in TTP, 27.3% in atypical HUS and 0% in snake bite. DISCUSSION Etiologic causes of TMA show regional differences. In our region, TMA group patients are frequently found and although it is espacially seen the most HELLP syndrome in etiology, TTP and snake bite are also common. In patients presenting with thrombocytopenia and MHA, TMA should be suspected and the etiology should be established as soon as possible. However, TPD treatment should be started as soon as possible because of the positive effect on mortality and morbidity without waiting for the definitive TMA etiology. Key words: Thrombotic microangiopathy, Therapeutic plasma exchange, TTP, HELLP syndrome, Snake bite, Atypical Hemolytic Uremic Syndrome
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Hematoloji, Hematology
