DOI: 10.36347/sjams.2017.v05i04.030

Pages: 1370-1373

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disorder of hematopoietic stem cells characterized by hemolytic anemia, marrow failure, and a high incidence of life-threatening thrombosis. PNH can be diagnosed with a single blood test and has a poor prognosis if untreated. It is an important cause of intra-abdominal thrombosis and therapy with the anti-complement drug, eculizumab, is very effective. Eculizumab usually prevents further thrombotic complications, thus emphasizing, that early diagnosis is critical. We report a case of a young female who presented with Budd Chiari syndrome and pancytopenia and on further evaluation turned out to be a case of PNH.