Red blood cell exchange followed by plasma exchange in patients with intrahepatic cholestasis due to sickle cell disease

İntrahepatik kolestaz, belirgin düzeyde artmış bilüribin ve akut hepatik yetmezlik ile karakterize, orak hücreli aneminin nadir bir komplikasyonu olup sıklıkla ölümcül gidişlidir. Bu raporda, intrahepatik kolestazı olan 2 homozigot hemoglobin S, bir S-? talesemi hastası bildirilmektedir. Otomatize eritrosit değişimi ile başarılı bir şekilde periferik kanda hemoglobin S düzeyi %30'un altına indirilmesine rağmen, kolestazın temel değerlendirme testlerinden olan bilüribin düzeyi birinci hastada 50 mg/dL, ikincisinde 30 mg/dL ve üçüncü hastada 10 mg/dL'nin üzerinde saptandı. Plazma değişimi işlemi uygulanan hastalardan biri dışında diğerlerinde klinik durumlarında iyileşme gözlendi. Bu vakalardan elde edilen veriler intrahepatik kolestazı olan orak hücreli anemi hastalarında eritrosit değişim işlemine yeterli yanıt alınamadığında plazma değişiminin klinik iyileşmede rol oynayabileceğini göstermektedir

Orakhücre hastalığına bağlıintrahepatik kolestazıolan hastalarda eritrosit değişiminin ardından plazma değişimi

Intrahepatic cholestasis, a rare complication of sickle cell anemia, is characterized by marked hyperbilirubinemia, acute hepatic failure, and an often fatal course. In this report, we present patients with homozygous hemoglobin S and one patient with sickle-? thalassemia disease who have intrahepatic cholestasis. Despite automated red blood cell exchange transfusion, which successfully decreased the hemoglobin S level to less than 30% of total hemoglobin level in the peripheral blood, basic biochemical laboratory examination revealed signs of cholestasis with a serum bilirubin level of up to 50 mg/dL in the first case, 30 mg/dL in the second, and 10 mg/dL in the third. The patients underwent plasma exchange, which improved their clinical condition except one. These cases suggest that plasma exchange may have a role in improving the clinical condition of patients with sickle cell disease complicated with intrahepatic cholestasis (SCIC) that fails to respond to automated red blood cell exchange therapy

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