Paroksismal nokturnal hemoglobinüride manyetik rezonans portografi bulguları
Paroksismal nokturnal hemoglobinüri (PNH) nadir görülen, akkiz bir he- matopoetik kök hücre hastalığıdır. İntravasküler hemoliz, kemik iliği yet- mezliği ve venöz trombozise eğilim söz konusu olup farklı klinik tablolar ile karşımıza çıkabilir. Hayatı tehdit edebilecek venöz trombozis atakla- rının tanısında ve takibinde radyolojik görüntüleme yöntemleri önem kazanmaktadır. Manyetik rezonans portografi günümüzde paroksismal nokturnal hemoglobinüri hastalarında abdominal venöz trombozis tara- masında ve takibinde sıklıkla kullanılmakta olup böbrekteki hemosiderin birikimini de ortaya koyabilmektedir.
MR portography findings in paroxysmal nocturnal hemoglobinuria
Paroxysmal nocturnal hemoglobinuria is a rare acquired hematopoet- ic stem cell disorder. It encompasses chronic intravascular hemolysis, bone marrow insufficiency, and recurrent venous thrombotic episodes. Patients may present with various clinical symptoms. Imaging studies are required for an adequate and prompt diagnosis of life threatening venous thrombosis episodes. Magnetic resonance portography is fre- quently used for the diagnosis and follow up of intraabdominal venous thrombosis in patients with paroxysmal nocturnal hemoglobinuria, and it also illustrates the hemosiderin deposition in the renal cortex.
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