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Thromb Haemost 1993; 70(02): 247-249
DOI: 10.1055/s-0038-1649478
Original Articles
Clinical Studies
Schattauer GmbH Stuttgart

Replacement Therapy with a Purified Protein C Concentrate during Initiation of Oral Anticoagulation in Severe Protein C Congenital Deficiency

V De Stefano
The Istituto Semeiotica Medica, Università Cattolica, Rome, Italy
,
S Mastrangelo
The Istituto Semeiotica Medica, Università Cattolica, Rome, Italy
,
H P Schwarz
1   The Immuno AG, Wien, Austria
,
P Pola
2   The Cattedra di Metodologia Clinica Medica, Università Cattolica, Rome, Italy
,
R Flore
2   The Cattedra di Metodologia Clinica Medica, Università Cattolica, Rome, Italy
,
B Bizzi
The Istituto Semeiotica Medica, Università Cattolica, Rome, Italy
,
G Leone
The Istituto Semeiotica Medica, Università Cattolica, Rome, Italy
› Author Affiliations
Further Information

Publication History

Received 25 August 1992

Accepted after revision 16 February 1993

Publication Date:
04 July 2018 (online)

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Summary

The case of an adult patient with moderately severe protein C deficiency (antigen 16%, activity 12%) is reported. Both parents had protein C levels compatible with heterozygous deficiency. Unlike other reported cases of severe protein C deficiency in adults, the onset of thrombotic symptoms occurred at 1 month of age; however, a symptom-free period until age 17 followed. Replacement therapy with a monoclonal antibody purified protein C concentrate was carried out during the initiation of oral anticoagulation after a course of i.v. heparin for deep vein thrombosis. The administration of the concentrate allowed maintenance of protein C above 50% until a stable therapeutic anticoagulation level could be obtained. This was reached within a short time, thus allowing safe administration of a loading dose of warfarin. We conclude that this approach to the prevention of skin necrosis seems more rapid and safer than previous schedules of oral anticoagulation in protein C-deficient patients.

 

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