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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  • EP ID EP372108
  • DOI -
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How To Cite

Sarabjot Kaur, Narendra Meena, Ravinder Garg, Sumit Pal Singh Chawla, Vidhi Singla (2017). Paroxysmal Nocturnal Hemoglobinuria Causing Budd Chiari Syndrome - A Rare Case Report. Scholars Journal of Applied Medical Sciences, 5(4), 1370-1373. https://europub.co.uk/articles/-A-372108