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.

CONCLUSION:

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: Hervé Mal, Agnès Veyradier, Olivier Brugière, Daniel Da Silva, Magali Colombat, Elie Azoulay, Laurent Benayoun, Eric Rondeau, Gaelle Dauriat, Camille Taillé, Guy Lesèche, Yves Castier, Michel Fournier
• Journal: Transplantation (Transplantation) Vol. 81 Issue 12 Pg. 1628-32 (Jun 27 2006) ISSN: 0041-1337 [Print] United States
• PMID: 16794526 (Publication Type: Case Reports, Journal Article)

Chemical References

• ADAM Proteins
• ADAMTS13 Protein
• ADAMTS13 protein, human

Topics

• ADAM Proteins (deficiency, genetics, metabolism)
• ADAMTS13 Protein
• Adult
• Biopsy
• Female
• Humans
• Lung Transplantation
• Male
• Middle Aged
• Thrombosis (pathology)