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Thromb Haemost 1997; 77(06): 1086-1089
DOI: 10.1055/s-0038-1656117
Clinical Studies
Schattauer GmbH Stuttgart

Purpura Fulminans Induced by Disseminated Intravascular Coagulation following Infection in 2 Unrelated Children with Double Heterozygosity for Factor V Leiden and Protein S Deficiency

Aida Inbal
1   The Institute of Thrombosis and Hemostasis, Department of Hematology, Sheba Medical Center, Tel-Hashomer and Sackler School of Medicine, Tel-Aviv University, France
,
Gilly Kenet
1   The Institute of Thrombosis and Hemostasis, Department of Hematology, Sheba Medical Center, Tel-Hashomer and Sackler School of Medicine, Tel-Aviv University, France
,
Ariella Zivelin
1   The Institute of Thrombosis and Hemostasis, Department of Hematology, Sheba Medical Center, Tel-Hashomer and Sackler School of Medicine, Tel-Aviv University, France
,
Tikva Yermiyahu
2   Institute of Haematology and Pediatrics, Soroka Medical Center, Beer-Sheva, France
,
Tamar Bronstein
2   Institute of Haematology and Pediatrics, Soroka Medical Center, Beer-Sheva, France
,
Tomy Sheinfeld
3   Schneider Children's Medical Center, Petach-Tiqva, Israel
,
Hana Tamari
3   Schneider Children's Medical Center, Petach-Tiqva, Israel
,
Sanford Gitel
1   The Institute of Thrombosis and Hemostasis, Department of Hematology, Sheba Medical Center, Tel-Hashomer and Sackler School of Medicine, Tel-Aviv University, France
,
Gill Eshel
4   Pediatric Intensive Care Unit, Assaf-Harofeh, Zrifin, Israel
,
Jerome Duchemin
5   INSERM Unité 428, Paris, France
,
Martine Aiach
5   INSERM Unité 428, Paris, France
,
Uri Seligsohn
1   The Institute of Thrombosis and Hemostasis, Department of Hematology, Sheba Medical Center, Tel-Hashomer and Sackler School of Medicine, Tel-Aviv University, France
› Author Affiliations
Further Information

Publication History

Received 30 July 1996

Accepted after resubmission 05 March 1997

Publication Date:
26 July 2018 (online)

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Summary

Purpura fulminans is associated with homozygous protein C and homozygous protein S deficiency or may follow bacterial or viral infections. We present 2 children from 2 unrelated Arab families with purpura fulminans who were double heterozygotes for factor V Leiden inherited from their fathers and protein S deficiency inherited from their mothers. No previous thrombotic events have occured in either patient or their respective family members. In one patient sepsis accompanied by disseminated intravascular coagulation appeared to be the trigger of purpura fulminans. In the other patient varicella infection preceded purpura fulminans and was also associated with disseminated intravascular coagulation. This report emphasizes the need for evaluation of hereditary defects in the inhibitory mechanisms of blood coagulation in patients with purpura fulminans at any age.

 

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