Introduction: Immune thrombocytopenic purpura (ITP) is an acquired disorder characterized by isolated thrombocytopenia. There is no "gold standard" test that can establish the diagnosis of primary ITP. Therefore, the diagnosis is one of exclusion and is based on patient history, physical examination, complete blood count, and blood smear review. Platelet indices are biomarkers of platelet activation, allowing extensive investigations focusing on the diagnostic and prognostic values in various diseases without extra-costs. Mean platelet volume (MPV), platelet distribution width (PDW) and plateletcrit (PCT) were investigated aiming at evaluating the correlation between pre-and post-treatment values in ITP patients. The sensitivity and specificity of platelet indices for assessment of their predictive value for treatment response were also evaluated. Methods: Fifty newly diagnosed primary ITP patients were selected from the Hematology Clinics of Ain Shams University Hospitals and classified into pediatric and adult groups. Platelet count, MPV, PDW, PCT have been recorded for all pre and post-treatment CBC samples using Beckman Coulter LH750 analyzer. Results: In pediatrics post-treatment MPV and PDW were significantly decreased while PCT was increased when compared to their values at the time of presentation (P < 0.01). The same results were obtained in adult patients, except for PDW which did not attain any significance. A significant correlation was found between pre-treatment values of these platelet indices and treatment response in pediatrics, while no significance was found between pre-treatment PDW and treatment response in adults. Conclusion: Increased pre-treatment MPV and PDW, and decreased PCT can provide diagnostic and predictive value for treatment response in ITP patients.
Background: The diagnosis of underlying myeloproliferative neoplasms (MPNs) is often problematic in patients with Budd Chiari syndrome (BCS). A clonal mutation in JAK2 tyrosine kinase (JAK2V617F) occurs in a high proportion of patients with MPNs and is of use in the characterization of occult MPNs in BCS. Aim of the work: Detection of JAK2 V617F mutation in patients with BCS and its value in detection of occult MPNs. Patients and Methods:This study was carried out on fifty seven newly diagnosed Budd Chiari syndrome patientswho were attending tropical department in Ain Shams University Hospitals during the period from July 2017 to July 2018.Detection of JAK2V617F mutation was done by real time polymerase chain reaction.Results: Out of the studied 57 BCS patients, JAK2 V617F mutation was detected in 12 patients (21.1%) {10 (83.3%) were heterozygous and 2(16.7%) were homozygous for mutation}, while 45 patients (78.9%) were negative.On comparing JAK2 V617F positive and negative groups, there was a highly statistically significant relation regarding MPNs diagnosis, where all JAK2 V617F positive patients were diagnosed as MPNs of whom 7 (58.3%) had overt presentation and 5(41.7%) had occult presentation, while in JAK2 V617F negative patients only 2 were diagnosed overt MPNs (p=0.001). Conclusion:In conclusion the JAK2 V617F mutation is an acquired mutation that can be used for diagnosis of latent MPNs presenting with thrombotic events, thus it is recommended to include JAK2 V617F gene analysis in the research panel for BCS patients.
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