Fatih YILMAZ, Mesut AYER, İdris İNCE, Aylin AYER

Hepatik Ven Trombozunun Nadir Bir Sebebi: Paroksismal Noktürnal Hemoglobinüri

ÖZET Paroksismal nokturnal hemoglobinüri (PNH, StrübingMarchiafavaMicheli sendromu) intravasküler hemoliz, hemoglobinüri ve sıklıkla ciddi venöz trombotik komplikasyonlara neden olan nadir edinsel klonal hemopoietik kök hücre hastalığıdır. PNHnin en önemli komplikasyonu özellikle batın içi venleri etkileyen trombotik olaylardır. Hepatik ven trombozu (Budd-Chiari sendromu) PNH hastalarında sık gözlenir. Burada ilk olarak bisitopeni ve splenomegali ile kliniğimize başvuran ve yatışı sırasında PNH tanısı koyulan bir olgu sunuyoruz.

Paroxysmal Nocturnal Hemoglobınurıa: A Rare Cause Of Hepatıc Veın Thrombosıs

Paroxysmal Nocturnal Hemoglobınurıa: A Rare Cause Of Hepatıc Veın Thrombosıs Paroxysmal nocturnal haemoglobinuria (PNH, StrübingMarchiafavaMicheli syndrome) is a rare acquired clonal disorder of haematopoietic stem cells causing intravascular haemolysis, haemoglobinuria and occasionally severe venous thrombotic complications. The most important complications of PNH, are thrombotic events, especially affecting intra-abdominal veins. Hepatic vein thrombosis (Budd-Chiari syndrome) is common in PNH patients. Herein, we present a case who was admitted to our clinic with bicytopenia, splenomegaly and diagnosed to have PNH during hospitalization.

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