Purpura fulminans in severe congenital protein C deficiency: monitoring of treatment with protein C concentrate.
This report describes the successful use of protein C concentrate to treat severe purpura fulminans in a homozygous protein C-deficient infant for 8 months until oral anticoagulation was initiated. While fresh frozen plasma was previously used in such cases to replace protein C in the acute phase, the availability of a monoclonal antibody purified protein C concentrate now allows specific replacement of protein C, avoiding problems of fluid overload. An occlusive-hydrocolloid bandage proved to be effective in local treatment of skin lesions. D-dimer, fibrin monomer, thrombin-antithrombin complex and prothrombin fragment 1 + 2 were useful markers in monitoring and optimizing protein C replacement therapy.


CONCLUSIONS
Diagnosis of protein C deficiency should be considered in a newborn with purpura fulminans. Early diagnosis and adequate replacement therapy is life-saving. Today, administration of protein C is the acute as well as long-term therapy of choice.
Müller
F M
FM
University Children's Hospital, Mainz, Germany.
Ehrenthal
W
W
Hafner
G
G
Schranz
D
D
D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D001458:Bandages; D001779:Blood Coagulation Factors / Q000378:metabolism; D003102:Colloids; D005260:Female; D005343:Fibrinolytic Agents / Q000627:therapeutic use; D006493:Heparin / Q000627:therapeutic use; D006801:Humans; D007231:Infant, Newborn; D010951:Plasma Exchange; D011486:Protein C / Q000378:metabolism / Q000627:therapeutic use*; D020151:Protein C Deficiency; D011693:Purpura / Q000150:complications / Q000209:etiology* / Q000628:therapy; D012163:Retinal Detachment / Q000209:etiology; D013924:Thrombophlebitis / Q000209:etiology
D000911:Antibodies, Monoclonal; D001779:Blood Coagulation Factors; D003102:Colloids; D005343:Fibrinolytic Agents; D011486:Protein C; D006493:Heparin