الفهرس 
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Abstract
Bone marrow failure is defined as a group of disorders characterized by inability of bone marrow to produce sufficient blood cells. These disorders of haematopoietic stem cells can involve either one cell line or all three haematopoietic linages (erythroid for RBCs, myloid for WBCs and megakaryocyte for platlets ) (Moore ,2019).
They can be divided into inherited and acquired disordeds .the most common inherited disorders are Fanconi anemia,shwashman-diamond syndrome,Diskeratosis congenita,Diamond-Blackfan anemia,severe congenital neutropenia(Alter,2010) but the acquired disorders mainly include Aplastic anemia and paroxysmal nocturnal hemoglobinuria and the diseases infiltrating bone marrow such as Gaucher disease, myelofibrosis and Malignancy.(Katherine , 2007).
More than 80 genetic mutations have been resulted in bone marrow failure development.participation of mutated gene products in housekeeping pathways (these pathways are essential for cell growth and division) results in disturbance of these pathways and activates p53 which causes cell cycle arrest.(Zang,2016).
Common symptoms of bone marrow failure are associated with inability of bone marrow to produce sufficient blood cells :deficiency of neutrophils results in bacterial infections,fevers ,deficiency of RBcs causes anemia symptoms and deficiency of platelets causes bleeding tendency .the first step in diagnosis is analysis of peripheral blood then, bone marrow aspirate and biopsy showing hypocellular bone marrow .(Moore ,2019)
Bone marrow failure syndromes are characterized by worse prognosis and elevation of cancer predisposition and deaths rate.The only cure of bone marrow failure syndromes stays hematopoietic stem cell transplantation.especially before complications development .(Calado,2017).
The aim of the work is to study the registery of cases and clinical spectrum of bone marrow failure syndromes in Beni-Suef University hospital.It was a descriptive study in which Patients with BMF were registered from the all pediatric group(0-12years) presented to pediatric out clinic ,hematology clinic and the admitted cases in the internal department and in the Neonatal intensive care unit in Beni-Suef university hospital in a duration of six months from January 2019 to June 2019.
Our findings were:55 patients diagnosed with bone marrow failure . of these patients in this registery: (21.8% had Fanconi anemia,5.5% had Diamond-Blackfan anemia,27.3% had idiopathic aplastic anemia,1.8% had secondary aplastic anemia due to CMV infection , 3.6% had paroxysmal nocturnal hemoglobinuria,30.9%had acute leukemia ,7.3% had Gaucher disease,1.8% had Myelofibrosis).
This report represent the first comprehensive hospitalized based study evaluating the prevalence and the clinical spectrum of the inherited,acquired bone marrow failure disorders. based on the data from this registry, the incidence of BMF was calculated to be approximately 5 cases per 2000 cases presented to pediatrician out clinic in Beni-Suef university hospital ,and 3.3% of admitted cases during 6 months duration to the internal department of Beni-Suef university hospital.
Based on that, we assumed that registery of occurrence of these disorders increased significantly However,further studies are required to detect new trials for early diagnosis ,consequently, new ways of prevention and treatment.