Evaluation of Haemostatic Mechanism in Children with Sickle Cell Anemia and Sickle Cell Trait at Gaffer Ibn Auf Children Hospital- Khartoum

Abstract

Sickle cell anemia is an autosomal recessive disorder characterized primarily by chronic anemia and periodic episodes of pain.The disorder produces abnormal hemoglobin,which causes the red blood cells to sickle or became crescent shaped.These sickle shaped RBCs are much less deformable,and therefore obstruct micro circulation,and cause tissue infraction . This study aimed to evaluate the hemostatic mechanism of patients with sickle cell anemia in comparison to healthy controls.The age range of this population was from 2-15 years. The sex percent of males 47.5% and females 52.5% . Venous blood samples were collected from patient with sickle cell anemia (n=40) and controls(n=10) attending Gaffar Ibn Aufe Ghildren Hospital,at Khartoum State.The samples of patients with sickle cell disease {SS=85%},Sickle cell trait {AS=15%}and 10 healthy controls{AA} were tested for prothrombin time {PT},activated partial thromboplastin {aPTT}and platelets count. The mean and standard deviation for hematological parameters for each group (SS,AS) were compared with that of control using SPSS. This study revealed significant differences only in platelets count of patient with sickle cell anemia during pain full cricess (SS) ( P>0.05) , comparing with control(AA ).The present study did not find any significant differences(P<0.05) between healthy controls and patient with sickle cell anemia in steady state{SS} and patient with sickle cell anemia trait { AS} ‫‪This study show the platelets found to be high (thrombocytosis) in patient‬‬ ‫‪with sickle cell anemia during painfull cricess (SS). The results of the‬‬ ‫‪present study concluded that sickle cell anemia is associated with‬‬ ‫‪thrombocytosis due to functional asplenia from the repetitive splenic‬‬ ‫.‪autoinfarcts‬‬ ‫‪This study recommended pre screening for infants and children as‬‬ ‫‪necessary precautions to avoid complication of sickle cell anemia that‬‬ ‫.‪may lead to death‬‬