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Protein C and Protein S: causative factor for developing a hemorrhagic infarct in a HbE/Beta thalassemia child

Sharma Vineeta1, Arijit Biswas, Bijender Kumar

  • 1Department of Hematology, All India Institute of Medical Sciences, New Delhi, India.

Indian Journal of Pediatrics
|January 22, 2010
PubMed

Insights

This study identifies Protein C and Protein S deficiency as the cause of hemorrhagic infarct in a child with HbE/Beta thalassemia. This finding highlights a crucial link between specific genetic mutations and coagulation disorders.

Area of Science:

  • Hematology
  • Neurology
  • Genetics

Background:

  • HbE/Beta thalassemia is a common hemoglobinopathy.
  • Hemorrhagic infarcts are rare in pediatric patients, especially those with hemoglobinopathies.
  • Understanding the etiology of infarcts in this population is critical for effective management.

Observation:

  • A 10-year-old child with HbE/Beta thalassemia presented with occipitotemporal hemorrhagic infarct and frontal white matter gliotic changes.
  • Genetic testing revealed HbE and IVS1-5 mutations, but no thrombogenic mutations.
  • Magnetic resonance imaging confirmed the hemorrhagic infarct.

Findings:

  • Low antigenic levels of Protein C and Protein S were detected in the patient.
  • Protein C and Protein S deficiency were identified as the causative factors for the hemorrhagic infarct.
  • The absence of thrombogenic mutations suggests a non-thrombotic pathway for infarct development.

Implications:

  • This case underscores the importance of evaluating coagulation factors in pediatric patients with HbE/Beta thalassemia presenting with infarcts.
  • Early diagnosis and management of Protein C and Protein S deficiency can prevent recurrent thromboembolic events.
  • Further research is warranted to explore the interplay between hemoglobinopathies and inherited thrombophilias.

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