Long-term Management of Homozygous Protein C Deficiency: Replacement Therapy with Subcutaneous Purified Protein C Concentrate

C. Sanz-Rodriguez; J. J. Gil-Fernández; P. Zapater; I. Pinilla; E. Granados; V. Gómez-G. de Soria; J. Cano; N. Sala; J. M. Fernández-Rañada; N. Gómez Gómez

doi:10.1055/s-0037-1614593

Thrombosis and Haemostasis, Table of Contents

Thromb Haemost 1999; 81(06): 887-890
DOI: 10.1055/s-0037-1614593

Letters to the Editor

Schattauer GmbH

Long-term Management of Homozygous Protein C Deficiency: Replacement Therapy with Subcutaneous Purified Protein C Concentrate

Authors

C. Sanz-Rodriguez

¹From the Departments of Hematology and Madrid, Spain
J. J. Gil-Fernández

¹From the Departments of Hematology and Madrid, Spain
P. Zapater

²Clinical Pharmacology, Hospital Universitario de la Princesa, Madrid, Spain
I. Pinilla

¹From the Departments of Hematology and Madrid, Spain
E. Granados

¹From the Departments of Hematology and Madrid, Spain
V. Gómez-G. de Soria

¹From the Departments of Hematology and Madrid, Spain
J. Cano

³Department of Neonatology, Hospital Niño Jesús, Madrid, Spain
N. Sala

⁴Medical and Molecular Genetics Center, Institut de Recerca Oncològica, Barcelona, Spain
J. M. Fernández-Rañada

¹From the Departments of Hematology and Madrid, Spain
N. Gómez Gómez

¹From the Departments of Hematology and Madrid, Spain

Abstract

Summary

We present the case of a full-term newborn in whom purpura fulminans developed shortly after birth. A diagnosis of homozygous protein C deficiency was established based upon undetectable plasma protein C activity and antigenemia in the newborn infant, and was later confirmed by protein C gene analysis. 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 with intravenous protein C concentrate. However, oral anticoagulation was finally abandoned as the patient presented several thrombotic and hemorrhagic episodes clearly related to difficulties with anticoagulation. Due to the hazards related to prolonged venous access, we are currently using subcutaneous infusion of protein C concentrate for the longterm management of this condition, with satisfactory results.

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References

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