PEDIATRICS Vol. 77 No. 5 May 1986, pp. 670-676
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Purpura Fulminans in a Chinese Boy With Congenital Protein C Deficiency

Patrick Yuen MD, FRCP(C)1, Alfred Cheung PhD1, Hsiang Ju Lin PhD1, Faith Ho MBBS, MRC(Path)1, Jun Mimuro MD1, Nobuhiko Yoshida MD1, and Nobuo Aoki MD1

1 From the Department of Pediatrics, Prince of Wales Hospital, The Chinese University of Hong Kong, Shatin, New Territories, and Department of Pathology, University of Hong Kong, Hong Kong; and Institute of Hematology and Department of Medicine, Jichi Medical School, Tochigi-Ken, Japan

Severe and recurrent purpura fulminans developed in a Chinese boy at one day of age. Results of coagulation studies performed on the patient during attacks were compatible with the diagnosis of disseminated intravascular coagulation. Subsequent investigations have revealed that the patient is homozygous and that his parents are heterozygous for protein C deficiency. Cryoprecipitate and fresh frozen plasma induced a remission, and administration of warfarin has been successful in preventing recurrence of attacks for as long as 8 months without infusion of any plasma components. None of the family members who are heterozygous for protein C deficiency have had thrombotic episodes.

Key Words: purpura fulminans • congenital protein C deficiency

Submitted on January 28, 1985
Accepted on June 26, 1985


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