Treatment of plasminogen deficiency patients with fresh frozen plasma

Kizilocak H., Ozdemir N., Dikme G., Koc B., Atabek A. A., Cokugras H., ...Daha Fazla

PEDIATRIC BLOOD & CANCER, cilt.65, sa.2, 2018 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 65 Sayı: 2
  • Basım Tarihi: 2018
  • Doi Numarası: 10.1002/pbc.26779
  • Dergi Adı: PEDIATRIC BLOOD & CANCER
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Anahtar Kelimeler: ligneous conjunctivitis, ligneous gingivitis, plasminogen, FEMALE GENITAL-TRACT, RISK-FACTOR, LIGNEOUS CONJUNCTIVITIS, THROMBOSIS, HYDROCEPHALUS, INFLAMMATION
  • Hacettepe Üniversitesi Adresli: Evet

Özet

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.