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Treatment of plasminogen deficiency patients with fresh frozen plasma
Author(s) -
Kızılocak Hande,
Ozdemir Nihal,
Dikme Gürcan,
Koç Begüm,
Atabek Ayşe Ayzıt,
Çokuğraş Haluk,
İskeleli Güzin,
DönmezDemir Buket,
Christiansen Nina Merete,
Ziegler Maike,
Ozdağ Hilal,
Schuster Volker,
Celkan Tiraje
Publication year - 2018
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.26779
Subject(s) - fresh frozen plasma , medicine , surgery , gastroenterology , platelet
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.