الفهرس 
Chronic isolated thrombocytopenia in children and adolescentsOnly 14 pages are availabe for public view
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Abstract
Background: The concept of chronic thrombocytopenia of childhood includes several acquired or inborn syndromes usually with a shortened lifespan of platelets in the peripheral blood; the most common one being idiopathic thrombocytopenic purpura (ITP). Objectives: Evaluation of children and adolescents with chronic isolated thrombocytopenia to identify uncommon causes of chronic isolated thrombocytopenia including chronic infections and genetic causes. Patients and methods: This cross-sectional study included children (1-18yrs) with chronic isolated thrombocytopenia who were regularly attending the Pediatric Hematology Clinic, Children’s Hospital, Ain Shams University. It included patients following up during the period from January 2020 through January 2024. Demographic data, family history, clinical and laboratory data were all recorded. Results: The study included 62 patients with equal male & female distribution and the mean age was 9.44 ± 3.84 SD years. The most common diagnosis was ITP (67.7%), followed by Bernard Soulier syndrome (9.7%), Glanzmann thrombasthenia (4.8%), chronic amegakaryocytic thrombocytopenia (4.8%), type 2B Von Willebrand disease (VWD) (4.8%), autoimmune lymphoproliferative syndrome (ALPS) (4.8%), one patient then developed other co-morbidities, lipopolysaccharide-responsive beige-like anchor (LRBA) (Genetically proven) (1.6%), thrombocytopenia absent radius (TAR) (1.6%). The family history of bleeding disorder was (41.9%) and recurrent infections (6.5%), diabetes mellitus (1.6%). The mean platelet count was 73.66 ± 34.42 x103/mm3 and the mean of MPV was 11.95 ± 2.02 fL Bone marrow examination was done in 47 cases (75.8%); (72.3%) of them showed peripheral platelet destruction suggesting ITP. Double negative T cell was performed in 8 cases, 3 of them (37.5%) showed positive results confirming the diagnosis of ALPS. The complete response to thrombopoietin-receptor agonists (TPO-RAs) was seen in (89.2%) and complete response to steroids was reported in (56.1%) of ITP cases. Conclusion: ITP is the most common cause of chronic thrombocytopenia in children, however highly structured diagnostic approach is required for detection of common causes of chronic thrombocytopenia including immune dysregulation disorders and inherited platelet defects.