INHERITED PROTEIN C DEFICIENCY AND COUMARIN-RESPONSIVE CHRONIC RELAPSING PURPURA FULMINANS IN A NEWBORN INFANT

Herman E. Branson*, Richard Marble, Jacob Katz, John H. Griffin

*Corresponding author for this work

Research output: Contribution to journalArticle

255 Scopus citations

Abstract

A coumarin-responsive chronic relapsing purpura fulminans syndrome is described in a protein-C-deficient newborn infant. Episodes of acute disseminated intravascular coagulation (DIC) and cutaneous gangrene, which first appeared at age 11 h, were effectively controlled for 28 months with transfusions of fresh-frozen plasma. Cryoprecipitate and cryoprecipitate-poor plasma induced remissions as long as those induced by fresh-frozen plasma (≤72 h). Coumarins sustained a cryoprecipitate- induced remission for 19 days: they were then electively discontinued and 17 h later the patient had an acute exacerbation of DIC with haemorrhaging. Family studies showed protein C levels of 31-40% in the subject's symptom-free mother and full and half brothers. DIC, the coumarin effect, and the inherited protein C abnormality appear to have contributed to the extremely low plasma levels (≤6%) of protein C in the patient. This experience suggests that protein C deficiency may greatly compromise the ability of newborn infants to control consumptive disorders.

Original languageEnglish (US)
Pages (from-to)1165-1168
Number of pages4
JournalThe Lancet
Volume322
Issue number8360
DOIs
StatePublished - Nov 19 1983

ASJC Scopus subject areas

  • Medicine(all)

Fingerprint Dive into the research topics of 'INHERITED PROTEIN C DEFICIENCY AND COUMARIN-RESPONSIVE CHRONIC RELAPSING PURPURA FULMINANS IN A NEWBORN INFANT'. Together they form a unique fingerprint.

  • Cite this