Abstract

Case Report

A fatal portal vein thrombosis: A case report

M Kechida*, W Ben Yahia and W Mnari

Published: 27 July, 2019 | Volume 3 - Issue 1 | Pages: 007-008

Background: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired hematologic condition which could be revealed by deep venous thrombosis. It could be fatal unless correctly treated.

Case report: We report here the case of a 28 year-old male with no medical history who was admitted to the emergency room for severe abdominal pain. Computerized Tomography angiography (CT) scan revealed portal vein thrombosis. Laboratory findings showed pancytopenia with severe regenerative normocytic anemia resulting in PNH. Because of the lack of Eculizumab, treatment was first based on curative anticoagulation until bone marrow transplant, with no success.

Conclusion: PNH remains a severe disease with bad prognosis unless treated with Eculizumab.

Read Full Article HTML DOI: 10.29328/journal.avm.1001009 Cite this Article Read Full Article PDF

Keywords:

Hemoglobinuria; Paroxysmal; Venous Thrombosis; Pancytopenia; Anemia; Hemolytic

References

  1. Takeda J, Miyata T, Kawagoe K, Iida Y, Endo Y, et al. Deficiency of the GPI anchor caused by a somatic mutation of the PIG-A gene in paroxysmal nocturnal hemoglobinuria. Cell. 1993; 73: 703-711. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/8500164
  2. Morag Griffin and Talha Munir. Management of thrombosis inparoxysmal nocturnal hemoglobinuria: a clinician’s guide. Ther Adv Hematol. 2017; 8: 119-126.PubMed: https://www.ncbi.nlm.nih.gov/pubmed/28246555
  3. Ageno W, Dentali F, De Stefano V, Barco S, Lerede T, et al. Clonal populations of hematopoietic cells with paroxysmal nocturnal hemoglobinuria phenotype inpatients with splanchnic vein thrombosis. Thromb Res. 2014; 133: 1052-1055. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/24731559
  4. Hill A, Kelly RJ, Hillmen P. Thrombosis in paroxysmal nocturnal hemoglobinuria. Blood. 2013; 121: 4985-4996. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/23610373
  5. de Latour RP, Mary JY, Salanoubat C, Terriou L, Etienne G, et al. Paroxysmal nocturnal hemoglobinuria: natural history of disease subcategories. Blood. 2008; 112: 3099-3106. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/18535202

Figures:

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