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Thalassemia major-- on the verge of bleeding or thrombosis?

Rahul Naithani1, Jagdish Chandra, Shashi Narayan

  • 1Department of Pediatrics, Kalawati Saran Children's Hospital, New Delhi, 110001, India. dr_rahul6@hotmail.com

Hematology (Amsterdam, Netherlands)
|March 9, 2006
PubMed

Insights

Children with beta-thalassemia major show significant hemostatic system alterations, increasing risks for bleeding and future blood clots. Early childhood assessment is crucial for identifying these predisposing factors.

Area of Science:

  • Hematology
  • Pediatric Medicine
  • Thrombosis Research

Background:

  • Thrombotic events are a known complication in adult thalassemia patients.
  • Understanding early hemostatic changes in children is vital for predicting future risks.

Purpose of the Study:

  • To assess hemostatic derangements in polytransfused children with beta-thalassemia major (beta-TM).
  • To identify potential predisposing factors for thrombotic and hemorrhagic events in early childhood.

Main Methods:

  • Evaluated 54 children with beta-TM and 30 controls.
  • Measured complete blood count, coagulation times (PT, aPTT), protein C, protein S, Antithrombin III (AT III), fibrinogen, d-dimer, serum iron, serum ferritin, and liver function tests.

Main Results:

  • 33.3% had thrombocytopenia, 40.7% prolonged PT, and 46.3% prolonged aPTT.
  • Low levels of Protein C (26.2%), Protein S (28.6%), and AT III (46.8%) were observed.
  • High serum ferritin levels correlated with prolonged PT and lower protein S, indicating altered coagulation and iron overload.

Conclusions:

  • Polytransfused children with beta-thalassemia exhibit significant hemostatic system alterations.
  • These alterations predispose children to both hemorrhagic manifestations and future thromboembolic events.
  • Early assessment and management are critical for mitigating risks in pediatric beta-thalassemia.
Abstract

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