الفهرس 
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Abstract
SUMMARY
Immune thrombocytopenia (ITP) is a heterogeneous disorder, which is characterized by anti-platelet autoantibody-mediated autoimmune response targeting megakaryocyte or platelet leading to impairment of platelet production or acceleration of platelet destruction, resultinginlowerplateletcountwhichmayhaveapropensitytobleeding.
ITP is caused by peripheral destruction of platelets mediated by immunological mechanisms. Yet, the exact mechanism of immune dysfunction is often unclear. There is uncertainty as to whether the primary insult is caused by abnormal B cells, T cells, or both. Moreover, reduced production of platelets or increased activation of phagocytes has been also proposed. One of the explanations underlying ITP is the breakdown of immune tolerance. Accordingly, a defect during development and maturation of lymphocytes can lead to autoimmunity against platelets. This defect may be at an early stage in the development of the thymus (central tolerance) or later in mature lymphocytes as a result of antigenic stimulation (peripheral tolerance).
During the stages of maturation in the thymus and bone marrow, the light and heavy chains of B-cell receptor, respectively, undergo modification. The breakage in the DNA strand forms the functional receptors (coding joint recombination sites) KRECs. KREC is circular episomic DNA, therefore it does not divide in the cell and do not appear in the daughter cells.
The current study was a case-control study that conducted on the pediatrics hematology outpatient clinic in Beni-Suef University hospital, during the period from May 2021 (after the approval of the ethical committee) to be November 2021. It included 50 patients having ITP (subdivided into three groups: active= 20 patients, remission= 12 patients, and chronic= 18 patients) and 50 healthy subjects as controls.
Fromtheanalysisofourdatawefoundthat:
There was no significant difference between both groups regarding their presence of family history, and blood transfusion. There were no cases or controls with history of drug intake, fever and platelets transfusion.
The history of consanguinity was higher in cases with no significance. There were 88% of cases ecchymosis, and 80% of cases with pallor,74% of cases with epistaxis,73.5% of cases with purpuraand 16% of cases bleeding gums.
There were 94% on oral steroids, 12% on immuran, 6% on rivolette, and 42% on IV steroids. There were 38% had no response, 28% had partial responses, and 34% had complete responses.
There was no significant difference between both groups regarding the RBCs but WBCs was significantly higher in cases than controls, but platelets were significantly higher in controls than cases.
There was no significant association between disease classification and KRECs marker in cases group
There was no significant association between response to ttt and KRECs marker in cases group
There was no significant association between sex and KRECs marker in each group
There was no significant linear correlation between the KRECs and age, RBCs, WBCs and PLT
The B cells immunological marker (KRECs) was significantly higher in cases than controls.
There was a significant role of KRECs marker in prediction of purpura at a cut off >1.80 with sensitivity 100%, specificity 98%, PPV 98% and NPV 100%.