Paroxysmal Nocturnal Hemoglobinuria Causing Budd Chiari Syndrome - A Rare Case Report
Journal Title: Scholars Journal of Applied Medical Sciences - Year 2017, Vol 5, Issue 4
Abstract
Abstract: 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. Keywords:paroxysmal nocturnal hemglobinuria, hemolytic anemia, thrombosis, Budd Chiari Syndrome
Authors and Affiliations
Sarabjot Kaur, Narendra Meena, Ravinder Garg, Sumit Pal Singh Chawla, Vidhi Singla
Keywords
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