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