CHANGES OF SOME HEMOSTATIC FACTORS IN CHILDREN WITH THALASSEMIC SYNDROMES

Abstract

The thalassemias are a heterogenous group of inherited disorders of hemoglobin synthesis, all characterized by the absence or reduced output of one or more of the globin chains of hemoglobin (Weatherall, 2001).

The hemostatic system in thalassemic and sickle thalassemic patients is altered. They were proved that thalassemic patients were sussceptible to haemorragic manifestations in the form of bruises, epistaxis even cerebral haemorrages.

This work was carried out to asses Protein C, Protein S and von Willibrand Factor levels in thalassemic children, splenectomized and non- splenectomized and correlate this to the clinical finding.

Thirty patients were involved in tl:lis work selected from those attending the Pediatric Hematology Clinic, Minoufiya University including: -Twenty patients of fJ -thalassemia major with a mean age of

(7.82 ± 3.32) years. They were eleven males and nine females, eleven of them were splenectomized and nine were non­ splenectomized. - Ten patients of sickle fJ- thalassemia with a mean age of

(8.60 ± 2.31) years. They were four males and six females, three of them were splenectomized and seven were non- splenectomized.