UCL logo

UCL Discovery

UCL home » Library Services » Electronic resources » UCL Discovery

Porto-pulmonary hypertension exacerbated by platelet transfusion in a patient with ADAMTS13 deficiency

Elias, JE; Mackie, I; Eapen, CE; Chu, P; Shaw, JC; Elias, E; (2013) Porto-pulmonary hypertension exacerbated by platelet transfusion in a patient with ADAMTS13 deficiency. Journal of Hepatology , 58 (4) pp. 827-830. 10.1016/j.jhep.2012.11.003.

Full text not available from this repository.

Abstract

We propose that porto-pulmonary hypertension (PPH) may arise as a consequence of deficiency of ADAMTS13 (a plasma metalloprotease that regulates von Willebrand factor size and reduces its platelet adhesive activity) and provide a clinical case history to support our hypothesis. A patient with non-cirrhotic intrahepatic portal hypertension (NCIPH), ulcerative colitis and celiac disease developed symptoms of PPH, which had advanced beyond levels which would have made her an eligible candidate for liver transplantation (mean pulmonary artery pressure (PAP) 49 mmHg). She was known to have severe ADAMTS13 deficiency, which we considered to be causative of, or contributory to her NCIPH. We postulated that increasing porto-systemic shunting associated with advancing portal hypertension would make the next encountered vascular bed, the lung, susceptible to the pathogenic process that was previously confined to the portal system, with pulmonary hypertension as its consequence. Her pulmonary artery pressures fell significantly during the next year on weekly replacement of plasma ADAMTS13 by infusions of fresh frozen plasma and conventional drug treatment of her pulmonary hypertension. Her pulmonary artery pressures had fallen to acceptable levels when, in response to platelet infusion, it rose precipitously and dangerously. The sequence strongly supports our hypothesis that PPH is a consequence of ADAMTS13 deficiency and is caused by platelet deposition in afferent pulmonary vessels. © 2012 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.

Type: Article
Title: Porto-pulmonary hypertension exacerbated by platelet transfusion in a patient with ADAMTS13 deficiency
DOI: 10.1016/j.jhep.2012.11.003
UCL classification: UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Cancer Institute
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Cancer Institute > Research Department of Haematology
URI: http://discovery.ucl.ac.uk/id/eprint/1404876
Downloads since deposit
0Downloads
Download activity - last month
Download activity - last 12 months
Downloads by country - last 12 months

Archive Staff Only

View Item View Item