title: Porto-pulmonary hypertension exacerbated by platelet transfusion in a patient with ADAMTS13 deficiency.
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 mm Hg). 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.
authors.affiliation: Department of Medicine, University Hospitals of Leicester, Leicester, UK.
authors.forename: Joshua E
authors.initials: JE
authors.lastname: Elias
authors.forename: Ian
authors.initials: I
authors.lastname: Mackie
authors.forename: Chundamannil E
authors.initials: CE
authors.lastname: Eapen
authors.forename: Patrick
authors.initials: P
authors.lastname: Chu
authors.forename: Jean C
authors.initials: JC
authors.lastname: Shaw
authors.forename: Elwyn
authors.initials: E
authors.lastname: Elias
mesh_terms: D051722:ADAM Proteins / Q000097:blood / Q000172:deficiency*; D000071120:ADAMTS13 Protein; D000328:Adult; D062186:Arterial Pressure; D002446:Celiac Disease / Q000150:complications; D003093:Colitis, Ulcerative / Q000150:complications; D005260:Female; D006333:Heart Failure / Q000209:etiology; D006801:Humans; D006975:Hypertension, Portal / Q000097:blood* / Q000209:etiology*; D006976:Hypertension, Pulmonary / Q000097:blood* / Q000209:etiology* / Q000503:physiopathology; D017713:Platelet Transfusion / Q000009:adverse effects*; D018497:Ventricular Dysfunction, Right / Q000209:etiology; D014841:von Willebrand Factor / Q000378:metabolism
chemical_list: D014841:von Willebrand Factor; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human