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; Gilly Kenet; Ariella Zivelin; Tikva Yermiyahu; Tamar Bronstein; Tomy Sheinfeld; Hana Tamari; Sanford Gitel; Gill Eshel; Jerome Duchemin; Martine Aiach; Uri Seligsohn

doi:10.1055/s-0038-1656117

Thrombosis and Haemostasis, Table of Contents

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

¹The Institute of Thrombosis and Hemostasis, Department of Hematology, Sheba Medical Center, Tel-Hashomer and Sackler School of Medicine, Tel-Aviv University, France

,

Gilly Kenet

¹The Institute of Thrombosis and Hemostasis, Department of Hematology, Sheba Medical Center, Tel-Hashomer and Sackler School of Medicine, Tel-Aviv University, France

,

Ariella Zivelin

¹The Institute of Thrombosis and Hemostasis, Department of Hematology, Sheba Medical Center, Tel-Hashomer and Sackler School of Medicine, Tel-Aviv University, France

,

Tikva Yermiyahu

²Institute of Haematology and Pediatrics, Soroka Medical Center, Beer-Sheva, France

,

Tamar Bronstein

²Institute of Haematology and Pediatrics, Soroka Medical Center, Beer-Sheva, France

,

Tomy Sheinfeld

³Schneider Children's Medical Center, Petach-Tiqva, Israel

,

Hana Tamari

³Schneider Children's Medical Center, Petach-Tiqva, Israel

,

Sanford Gitel

¹The Institute of Thrombosis and Hemostasis, Department of Hematology, Sheba Medical Center, Tel-Hashomer and Sackler School of Medicine, Tel-Aviv University, France

,

Gill Eshel

⁴Pediatric Intensive Care Unit, Assaf-Harofeh, Zrifin, Israel

,

Jerome Duchemin

⁵INSERM Unité 428, Paris, France

,

Martine Aiach

⁵INSERM Unité 428, Paris, France

,

Uri Seligsohn

¹The Institute of Thrombosis and Hemostasis, Department of Hematology, Sheba Medical Center, Tel-Hashomer and Sackler School of Medicine, Tel-Aviv University, France

› Author Affiliations

Abstract

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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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References

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