Homozygous protein C deficiency presenting as neonatal purpura fulminans: management with fresh frozen plasma, low molecular weight heparin and protein C concentrate


Kizilocak H. , Ozdemir N. , Dikme G. , Koc B., Celkan T.

JOURNAL OF THROMBOSIS AND THROMBOLYSIS, cilt.45, ss.315-318, 2018 (SCI İndekslerine Giren Dergi) identifier identifier identifier

  • Cilt numarası: 45 Konu: 2
  • Basım Tarihi: 2018
  • Doi Numarası: 10.1007/s11239-017-1606-x
  • Dergi Adı: JOURNAL OF THROMBOSIS AND THROMBOLYSIS
  • Sayfa Sayıları: ss.315-318

Özet

Purpura fulminans in neonates is a rapidly progressive thrombotic disorder manifesting as hemorrhagic skin infarction and disseminated intravascular coagulation. Being inherited in an autosomal dominant manner, it is a medical emergency. Clinical presentations of patients may vary depending on the genetic mutations. Retinal and intracranial hemorrhages are the worst clinical scenarios with persistent morbidity. During acute phase, fresh frozen plasma, protein C concentrates and anticoagulant therapy should be administered rapidly. Here we report a patient with homozygous protein C deficiency.