الفهرس 
IntroductionOnly 14 pages are availabe for public view
Abstract
Summary
utoimmune cytopenias are characterized by the
production of autoantibodies against differentiated
hematopoietic cells because of defects in central and/or
peripheral tolerance. It includes autoimmune hemolytic
anemia (AIHA), immune thrombocytopenic purpura (ITP),
autoimmune proliferative syndrome (ALPS) and Evans
syndrome (EV).
Cytotoxic T lymphocyte associated antigen-4 (CTLA-4)
is a costimulatory receptor that has a potent inhibitory signal
on antigen-activated immune responses and was detected in
many autoimmune disorders.
A native soluble form of CTLA-4 (sCTLA-4) has been
found to interfere with normal immune response leading to
auto-reactivity. It was observed in high levels in different
autoimmune diseases such as systemic lupus erythematosus,
autoimmune thyroid disease, celiac disease and systemic
sclerosis. The association between levels of sCTLA-4 and
autoimmune cytopenias of children and adolescents patients
such as AIHA, ITP, ALPS and ES has not investigated through
literature.
The aims of our study were to compare levels of soluble
CTLA-4 in different types of immune cytopenias and their
control in children and adolescent patients and to determine
the possible relation between sCTLA-4 and the disease
severity and response to treatment in this group of patients.
A
Summary
84
Forty seven children and adolescents who have
autoimmune cytopenias were recruited and assessed for
eligibility in Pediatric Hematology Clinic, Ain Shams
University Children‘s Hospital and forming a patients group
with age range 8 – 204 months old. An age and sex matched
healthy control group were recruited including forty seven
healthy participants with age range 6 – 156 months old.
Both patients and control groups were subjected to full
history taking, general examination and laboratory evaluation.
We also obtained the initial laboratory examinations and the
treatment protocols of the patients groups.
Our results showed upon comparison of patient group to
control group that patient group have significantly higher
levels of sCTLA4 and no significant differences between the
ITP, AIHA and ALPS patients regarding the levels of
measured sCTLA-4.
Our results revealed that there was no correlation between
the age at the study entry of the autoimmune cytopenic patients
and levels of sCTLA-4 (R= -0.028, P= 0.853).
Our results showed that sCTLA-4 is inversely related to
the platelet count (R= -0.324, P<0.05) and moreover, there
was a highly significant negative correlations between level
of sCTLA-4 and both hepatosplenomegaly and recurrent
infections and significant correlation between level of
sCTLA-4 and treatment protocol (steroids+ steroid sparing).
Summary
85
There was a highly significant differences in initial and
current laboratory platelets level among ITP, AIHA and
ALPS patients (P <0.05). Its level was the highest among
AIHA patients and ALPS patients in compare with ITP.
Our findings revealed a significant differences in the
prevalence of hepatosplenomegaly among ITP, AIHA and
ALPS patients (P <0.05) with higher prevalence among
ALPS patients.
There was a significant difference in the prevalence of
cardiac manifestations among autoimmune cytopenias
patients with a higher prevalence among AIHA patients in
compare with ITP and ALPS patients.
Our results revealed that the rate of recurrent infection
among ALPS and AIHA patients were high in comparing
with ITP patient