RT Journal Article
SR Electronic
T1 Inhalation of activated recombinant factor VII to treat pulmonary hemorrhage in a patient with cystic fibrosis
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP p2041
VO 38
IS Suppl 55
A1 Constantin Marcu
A1 Robert Bals
A1 Andreas Groeschel
A1 Christian Lensch
A1 Julia Woerner
A1 Sebastian Faendrich
YR 2011
UL http://erj.ersjournals.com/content/38/Suppl_55/p2041.abstract
AB Case: The patient (30y.o., female) was diagnosed with cystic fibrosis as child. Currently, she was hospitalized with fever and bilateral pulmonary infiltrates. She rapidly deteriorated to respiratory failure with the need of extracorporale membrane oxygenation (ECMO).Diffus pulmonary haemorrhage complicated the course on ECMO. After 2 days of unremitting bleeding with respiratory failure we used rFVIIa as an intervention of last resort. The patient was treated with inhalation of 50 μg/kg rFVIIa in 5 ml of sodium chloride via a jet-driven nebulizer, which was repeated after 24 hours. Hemorrhage was visualized bronchoscopically, and its resolution following the treatment was immediate and did not occur again.Discussion: Factor VII initiates clot formation by its interaction with TF. The FVIIa-TF complex activates factor X. Activated factor X activates prothrombin to thrombin, which converts fibrinogen to fibrin. Tissue factor is expressed in the lung alveoli during inflammation and therefore pulmonary administration of human recombinant activated factor VIIa (rFVIIa) could be a rational treatment option.Our report indicates the applicability of topical rFVIIa to control unremitting pulmonary haemorrhage. By limiting inhalation to the target organ, the therapeutic effect could be superior as a result of higher topical concentrations without systemic exposure. This could also reduce the risk of thrombotic complications reported with systemic administration of rFVIIa as well as clotting in the ECMO membrane.Conclusion: Intrapulmonary administration of activated recombinant factor VII (rFVIIa) via a jet-driven nebulizer may be an option in pulmonary haemorrhage.