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Treatment of plasminogen deficiency patients with fresh frozen plasma

Authors :
Maike Ziegler
Ayse Ayzit Atabek
Haluk Cokugras
Tiraje Celkan
Buket Dönmez-Demir
Guzin Iskeleli
Hilal Özdağ
Nina M. Christiansen
Volker Schuster
Gürcan Dikme
Hande Kizilocak
Nihal Özdemir
Begum Sirin Koc
Source :
Pediatric bloodcancer. 65(2)
Publication Year :
2017

Abstract

Congenital plasminogen (Plg) deficiency leads to the development of ligneous membranes on mucosal surfaces. Here, we report our experience with local and intravenous fresh frozen plasma (FFP). We retrospectively reviewed medical files of 17 patients and their eight first-degree relatives. Conjunctivitis was the main complaint. Thirteen patients were treated both with intravenous and conjunctival FFP. Venous thrombosis did not develop in any. Genetic evaluation revealed heterogeneous mutations as well as polymorphisms. Diagnosis and treatment of Plg deficiency is challenging; topical and intravenous FFP may be an alternative treatment.

Details

ISSN :
15455017
Volume :
65
Issue :
2
Database :
OpenAIRE
Journal :
Pediatric bloodcancer
Accession number :
edsair.doi.dedup.....244cca7ad303ead29f013b6d4536e571