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Hemofagositik Lenfohistiositoz Gelişen Bir Atipik Hemolitik Üremik Sendrom Olgusu

An Unusual Patient with Atypical Hemolytic Uremic Syndrome Who Developed Hemophagocytic Lymphohistiocytosis

Journal Name:

  • Türk Nefroloji, Diyaliz ve Transplantasyon Dergisi

Publication Year:

  • 2013
DOI: 
10.5262/tndt.2013.1002.18

Key Words:

Keywords (Original Language):

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Abstract (2. Language): 
Hemolytic uremic syndrome (HUS) is characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. Atypical HUS is characterized by the absence of antecedent diarrhea, tendency to relapse, a positive family history and poor therapeutic outcome. Here we report an 8-year-old boy who presented with atypical HUS and did not have antecedent diarrhea or infection. He developed prolonged fever unresponsive to broad-spectrum antibiotics with markedly elevated liver enzymes and hepatosplenomegaly. There were phagocytized macrophages in his bone marrow aspiration. Based on these observations and other laboratory fi ndings, he was diagnosed with hemophagocytic lymphohistiocytosis. He was successfully treated with plasma exchanges and low dose oral steroids. To our knowledge, this is the fi rst case of atypical HUS in the literature associated with hemophagocytic lymphohistiocytosis.
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Abstract (Original Language): 
Hemolitik üremik sendrom (HÜS) mikroanjiopatik hemolitik anemi, trombositopeni ve akut böbrek yetmezliği ile karakterize bir tablodur. Atipik HÜS’te başlangıçta diare olmayabilir, hastalığın tekrarlama eğilimi vardır, ailede benzer olgular olabilir ve prognoz kötüdür. Burada 8 yaşında atipik HÜS tablosu ile gelen 8 yaşında bir olgu sunuldu. Hastalığın seyrinde belirgin hepatospleneomegali ile birlikte karaciğer enzimlerinde belirgin artış ve geniş spektrumlu antibiyotiklerle düşmeyen ateş tablosu gelişti. Kemik iliğinde fagositoz yapmış makrofajlar görüldü. Diğer laboratuar bulguları ile birlikte hasta hemofagositik lenfohistiositoz tanısı aldı. Hastaya düşük doz oral steroid tedavisi ile birlikte plazmaferez tedavisi uygulandı.Bu olgu atipik HÜS ve hemofagositik lenfohistiositozun son derece nadir birlikteliğini vurgulamak amacı ile sunuldu..
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  • Turkish

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