C. Sanz-rodriguez et al., Long-term management of homozygous protein C deficiency: Replacement therapy with subcutaneous purified protein C concentrate, THROMB HAEM, 81(6), 1999, pp. 887-890
We present the case of a full-term newborn in whom purpura fulminans develo
ped shortly after birth. A diagnosis of homozygous protein C deficiency was
established based upon undetectable plasma protein C activity and antigene
mia in the newborn infant, and was later confirmed by protein C gene analys
is. Specific replacement therapy with intravenous protein C concentrate was
started 9 days after birth. This rapidly led to the complete regression of
cutaneous lesions and consumption coagulopathy. After stabilization, oral
anticoagulation was initiated in association with prophylactic treatment wi
th intravenous protein C concentrate. However, oral anticoagulation was fin
ally abandoned as the patient presented several thrombotic and hemorrhagic
episodes clearly related to difficulties with anticoagulation. Due to the h
azards related to prolonged venous access, we are currently using subcutane
ous infusion of protein C concentrate for the long term management of this
condition, with satisfactory results.