Kızılocak Hande, Ozdemir Nihal, Dikme Gürcan, Koç Begüm, Atabek Ayşe Ayzıt, Çokuğraş Haluk, İskeleli Güzin, Dönmez-Demir Buket, Christiansen Nina Merete, Ziegler Maike, Ozdağ Hilal, Schuster Volker, Celkan Tiraje
Department of Pediatric Hematology-Oncology, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey.
Department of Pediatric Pulmonology, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey.
Pediatr Blood Cancer. 2018 Feb;65(2). doi: 10.1002/pbc.26779. Epub 2017 Sep 6.
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.
先天性纤溶酶原（Plg）缺乏会导致黏膜表面形成木样膜。在此，我们报告我们使用局部和静脉注射新鲜冰冻血浆（FFP）的经验。我们回顾性地查阅了17例患者及其8名一级亲属的病历。结膜炎是主要症状。13例患者接受了静脉和结膜下FFP治疗。无一例发生静脉血栓形成。基因评估显示存在异质性突变以及多态性。Plg缺乏的诊断和治疗具有挑战性；局部和静脉注射FFP可能是一种替代治疗方法。
