الفهرس 
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Abstract
S
ickle cell anemia is associated with hypoxia-induced polymerization of the abnormal Hb S molecule, followed by RBC sickling, with resultant micro vascular occlusion (vasoocclusion) clinically manifested most often as an acute painful episode or ”crisis”, but with many other acute and chronic complications.
Vaso-occlusive crisis is the most common complication of SCA. The most common complaint is pain, and recurrent episodes may cause irreversible organ damage. One of the most severe forms is the ACS which occurs as a result of infarction of the lung parenchyma. This can rapidly result in death. Other types of vaso-occlusive crisis in SCA include dactylitis, priapism, abdominal pain and jaundice.
In this study we aimed at determining the plasma concentration of the fibrinolytic protein D-dimer and the inflammatory marker hs-CRP in SCA patients in steady state and VOC in addition to evaluating the clinical role of them in disease progression encountered in those patients. This will help in predicting VOC which is the most prevalent complication of SCD.
This study was conducted on 40 SCA patients who were admitted into Children’s Hospital, Ain Shams University. They were 26 males and 14 females. Their age ranged from 5 to 14 years. They were divided as following; 40 patients (steady state group) and 20 patients from them who developed VOC during the course of disease (crisis group). Twenty age and sex matched healthy individuals were included as control group.
In our work, on comparing different study groups (controls, steady state group who didn’t develop crisis, steady state group developed crisis, crisis group) as regard hs-CRP levels, we found that it was higher in crisis group than steady state groups and both of them were higher than control group. We also found that it was higher in steady state group that developed crisis than the group that didn’t develop crisis.
The current study demonstrated a significant increase in the D-dimer levels of SCA patients in the steady state, when compared to controls. There was also an approximate 2-3 folds increase in the D-dimer levels in the same patients who developed VOC. This confirms the activation of coagulation and fibrinolytic systems in patients with SCA in the steady state which is further elevated in VOC episodes.
This study was also designed to look at the association between clinical severity and persistent hepatomegaly in SCA subjects in order to determine a possible role for hepatomegaly as an index of severity. Hepatomegaly was found in 25 out of 40 (62.5%) in SCA patients. There was a strong positive correlation between hepatomegaly in SCA patients and clinical severity of the disease represented in hs-CRP, Plt count, D-dimer, WBCs count and the frequency of thrombotic attacks while there was a negative correlation with Hb which suggests that patients with persistent hepatomegaly have a more severe clinical course than those without, and clinicians managing them may have to pay more particular attention to them.
We had got also information about the association between frequency of VOC episodes and our studied markers. There was a strong positive correlation with hs-CRP, D-dimer, WBCs count and Plt count while there was negative correlation with Hb.