Cerebral Blood Flow Velocity, protein C & Protein S evaluation in Patients with β-Thalassemia Intermedia Using Transcranial Doppler Sonography

Abstract

SUMMARY T his case control study was conducted in hematology clinic-children hospital ain Shams University in the period from May 2015 to September 2015. This study was designed to evaluate transcranial arterial mean flow velocities using transcranial doppler and Protein C and S status in patients with beta-thalassemia intermedia compared to healthy controls as predictors for higher risk of silent cerebral ischemic events in these patients. This study included thirty patients with thalassemia intermedia. They were compared tso (30) healthy control with age ranging between 6-15 years old of the two studied groups. Written informed consent was obtained from all the study group (patients and control) or their first-degree families. Inclusion criteria:  Patients with thalassemia intermedia (diagnosed by onset of anemia, features of hemolysis on peripheral smear).  Age of the patients from 5-16 years old. Exclusion criteria:  Patients with neurological or gross cognitive signs or symptoms or both.  Patients with thrombosis.  Diabetes mellitus.  Patients with hypertension.  Patients with ischemic heart.  Previous cerebrovascular disease.  Sickle cell anemia. All patients included in this study were subjected to full history taking. 1. Thorough clinical examination laying stress on:  Assessment of anthropometric measures and demographic data (name, age and sex).  Age at diagnosis and the duration of the disease.  History of transfusion (age at first transfusion and transfusion index).  Iron chelation therapy (age at start, type, dose and compliance).  Hydroxyurea treatment (age at the start, type, dose, duration and compliance).  Hospital admission and history of complication. 2. Full clinical examination:  General examination (weight and height standard deviation and body mass index).  Systemic examination (CNS, chest, heart, abdomen). 3. Laboratory investigations:  Complete blood picture.  Serum ferritin (mean value over the last year of follow up): as a marker of iron stores.  Liver function test (ALT, AST, total and direct billirubin).  Serum level of protein C and S Radiological investigations: Transcranial Doppler study through transtemporal window at different depth range to assess mean flow velocities of middle cerebral arteries, anterior cerebral arteries, posterior cerebral arteries and basilar artery changes compared to its normal values for age. Our results revealed that the studied group of patients have lower values of the measured protein C and protein S of the control group. There was a significant difference between patients and the control group as regards protein C (mean value=79.25 ± 11.2 % in patients, 80.47± 7.33 % in contols); and protein S (mean value=84.19 ± 10.23 % in patients, 89.84 ± 16.35% in controls). Their levels were lower among our patients than controls. As regard transcranial doppler results; there were a signficantly higher blood flow velocities among patients withβ-TI who were not regularly transfused,not taking HU,not taking iron chelation compared to patients who were regularly transfused, taking HU and iron chelation There is significant positive correlation between RT.ACA mean blood flow velocity and platelet count{ P (0.039)}.