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Published on: September 9, 2012
[Protein C deficiency in patient with sepsis-associated disseminated intravascular coagulation and deep vein
Mauro Tassin1, Pablo Llarena, Federico Laffaye
1Terapia Intensiva Pediátrica, Hospital Provincial de Neuquén Dr. Eduardo Castro Rendón. mtassin@hospitalneuquen.org.ar
Insights
Disseminated intravascular coagulation (DIC), often caused by sepsis in children, involves abnormal blood clotting and factor consumption. This case reveals a concurrent deep vein thrombosis and inherited protein C deficiency in a patient with sepsis-associated DIC.
Area of Science:
- Hematology
- Pediatric critical care
- Pathophysiology
Background:
- Disseminated intravascular coagulation (DIC) is a serious condition characterized by abnormal activation of coagulation, leading to microvascular thrombosis and consumption of clotting factors and platelets.
- Sepsis is a primary driver of DIC in pediatric populations, presenting with diverse clinical manifestations including bleeding, purpura, and gangrene.
- Deep venous thrombosis (DVT) can be a complication of DIC, highlighting the complex interplay of prothrombotic and consumptive processes.
Observation:
- A pediatric patient presented with clinical signs of sepsis-associated DIC.
- Concurrent deep venous thrombosis was identified in the affected patient.
- Diagnostic workup revealed an underlying inherited thrombophilia, specifically protein C deficiency.
Findings:
- The patient's sepsis-associated DIC was complicated by deep venous thrombosis.
- Protein C deficiency was identified as a significant inherited thrombophilic factor.
- This case underscores the potential for coexisting thrombotic and consumptive coagulopathies in sepsis.
Implications:
- Early recognition of inherited thrombophilia is crucial in pediatric patients with sepsis-induced DIC and thrombosis.
- Understanding the genetic predisposition to thrombosis can guide management strategies for DIC.
- This case highlights the importance of comprehensive diagnostic evaluation in complex pediatric coagulation disorders.
Abstract:
Disseminate intravascular coagulation (DIC) is a clinical pathological syndrome associated to several diseases. Sepsis is the most common cause in infants and children. DIC results from the anomalous activation of blood coagulation, widespread formation of thrombi in the microcirculation, and consumption of clotting factors and platelets. Clinical findings are variable; the most common is bleeding, followed by purpura and acral gangrene (purpura fulminans). We report a patient with sepsis associated-DIC and concurrent deep venous thrombosis. The diagnostic evaluation allowed to discover inherited thrombophilia associated to protein C deficiency.
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