Thrombotic microangiopathy with acquired deficiency in ADAMTS 13 activity in lung transplant recipients.
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.
Mal
Hervé
H
Groupe de Transplantation Pulmonaire, Hôpital Beaujon, Assistance Publique Hx de Paris, Clichy, France. herve.mal@bjn.ap-hop-paris.fr
Veyradier
Agnès
A
Brugière
Olivier
O
Da Silva
Daniel
D
Colombat
Magali
M
Azoulay
Elie
E
Benayoun
Laurent
L
Rondeau
Eric
E
Dauriat
Gaelle
G
Taillé
Camille
C
Lesèche
Guy
G
Castier
Yves
Y
Fournier
Michel
M
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*
D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human