title: Thrombotic microangiopathy with acquired deficiency in ADAMTS 13 activity in lung transplant recipients.
abstract: BACKGROUND
  Thrombotic microangiopathy may develop after solid organ transplantation, but it is usually not associated with severe deficiency in von Willebrand factor-cleaving metalloprotease (ADAMTS 13) activity.
  
  
  METHODS
  We present the cases of two lung transplant recipients who experienced a thrombotic microangiopathy associated with an acquired severe (<5%) deficiency in ADAMTS 13 activity.
  
  
  RESULTS
  A major feature of both cases was the occurrence of a diffuse alveolar hemorrhage.
  
  
  CONCLUSIONS
  Our two cases of lung transplant patients, with thrombotic microangiopathy related to an acquired ADAMTS 13 deficiency recipients, confirm that this mechanism may also be involved in the pathogenesis of thrombotic microangiopathy developing after solid organ transplantation. Therefore, we consider that ADAMTS 13 activity should be assessed on a systematic basis in this setting.
authors.affiliation: Groupe de Transplantation Pulmonaire, Hôpital Beaujon, Assistance Publique Hx de Paris, Clichy, France. herve.mal@bjn.ap-hop-paris.fr
authors.forename: Hervé
authors.initials: H
authors.lastname: Mal
authors.forename: Agnès
authors.initials: A
authors.lastname: Veyradier
authors.forename: Olivier
authors.initials: O
authors.lastname: Brugière
authors.forename: Daniel
authors.initials: D
authors.lastname: Da Silva
authors.forename: Magali
authors.initials: M
authors.lastname: Colombat
authors.forename: Elie
authors.initials: E
authors.lastname: Azoulay
authors.forename: Laurent
authors.initials: L
authors.lastname: Benayoun
authors.forename: Eric
authors.initials: E
authors.lastname: Rondeau
authors.forename: Gaelle
authors.initials: G
authors.lastname: Dauriat
authors.forename: Camille
authors.initials: C
authors.lastname: Taillé
authors.forename: Guy
authors.initials: G
authors.lastname: Lesèche
authors.forename: Yves
authors.initials: Y
authors.lastname: Castier
authors.forename: Michel
authors.initials: M
authors.lastname: Fournier
mesh_terms: D051722:ADAM Proteins / Q000172:deficiency* / Q000235:genetics / Q000378:metabolism; D000071120:ADAMTS13 Protein; D000328:Adult; D001706:Biopsy; D005260:Female; D006801:Humans; D016040:Lung Transplantation; D008297:Male; D008875:Middle Aged; D013927:Thrombosis / Q000473:pathology*
chemical_list: D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human