Transcranial Doppler ultrasonography in patients with hemoglobinopathy: An experience from a tertiary center

OBJECTIVE Patients with hemoglobinopathy are prone to cerebrovascular event. Our aim was to screen the peak systolic flow velocity (PSV) using transcranial Doppler ultrasonography (TCD) in terms of cerebrovascular event risk in patients with beta thalassemia (β-thal) and sickle cell anemia (SCA). METHODS PSV and resistive index (RI) values were determined at internal carotid artery (ICA) and middle cerebral artery (MCA)-from both temporal regions using TCD. RESULTS A total of 55 participants (40 patients and 15 healthy people) were included in the study. Thirty-three (60%) of the participants were female. Among 40 patients, 12 patients (30%) had NTDT, 14 patients (35%) had SCA, and 14 patients (35%) had TDT diagnosis. Bilateral ICA and MCA were open in all patients and had a normal flow pattern. PSV and RI were not significantly different between study and control groups in right and left MCA and ICA. Patients with high platelet level (>450.000/mm3) had significantly higher PSV values in right MCA (96 vs.70 cm/s, p=0.05). Among patients with TDT, age of starting iron chelation and right ICA PSV values was significantly negatively correlated (r=-0.56; p=0.04). Clinical symptoms (headache and pain crisis), hydroxyurea, and chelation therapy did not effect PSV values. CONCLUSION Platelet level and age of starting iron chelation might be an influencing factor for PSV. Regular follow-up of patients, appropriate therapy and lack of other factors causing cerebrovascular events might be possible reason for these acceptable results.

[1]  M. Kraut,et al.  American Society of Hematology 2020 guidelines for sickle cell disease: prevention, diagnosis, and treatment of cerebrovascular disease in children and adults. , 2020, Blood advances.

[2]  S. Perrotta,et al.  An Educational Study Promoting the Delivery of Transcranial Doppler Ultrasound Screening in Paediatric Sickle Cell Disease: A European Multi-Centre Perspective , 2019, Journal of clinical medicine.

[3]  E. Akl,et al.  2019 sickle cell disease guidelines by the American Society of Hematology: methodology, challenges, and innovations. , 2019, Blood advances.

[4]  F. Esposito,et al.  No increased cerebrovascular involvement in adult beta-thalassemia by advanced MRI analyses. , 2019, Blood cells, molecules & diseases.

[5]  Naomi S. Bardach,et al.  Barriers to Pediatric Sickle Cell Disease Guideline Recommendations , 2019, Global pediatric health.

[6]  R. Adams,et al.  Ischemic stroke in children and young adults with sickle cell disease in the post‐STOP era , 2015, American journal of hematology.

[7]  J. Lipton,et al.  Erythropoiesis: insights into pathophysiology and treatments in 2017 , 2018, Molecular Medicine.

[8]  F. Kirkham,et al.  Central nervous system complications and management in sickle cell disease. , 2016, Blood.

[9]  R. Ware,et al.  Prevention of conversion to abnormal transcranial Doppler with hydroxyurea in sickle cell anemia: A Phase III international randomized clinical trial , 2015, American journal of hematology.

[10]  I. Papassotiriou,et al.  Evaluation of Intracranial Cerebral Blood Flow Velocities in Splenectomised and Non-Splenectomised Patients with β-Thalassemia Intermedia Using Transcranial Doppler Sonography. , 2015, In vivo.

[11]  Peter B. Soh,et al.  Changes in Cerebral Blood Flow in Children with Sickle Cell Disease After Splenectomy , 2015, Pediatric hematology and oncology.

[12]  E. Meier,et al.  Cerebral vasculopathy in children with sickle cell anemia. , 2015, Blood cells, molecules & diseases.

[13]  William J Savage,et al.  Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. , 2014, JAMA.

[14]  P. Petramfar,et al.  Cerebral Artery Velocity Determined by Transcranial Doppler Ultrasonography in Patients With β-Thalassemia Intermedia Compared to β-Thalassemia Major , 2013, Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis.

[15]  P. Petramfar,et al.  Intracranial Blood Flow Velocity in Patients with β-Thalassemia Intermedia Using Transcranial Doppler Sonography: A Case-Control Study , 2011, Anemia.

[16]  E. Kohne Hemoglobinopathies: clinical manifestations, diagnosis, and treatment. , 2011, Deutsches Arzteblatt international.

[17]  M. Cappellini,et al.  Mediterranean Journal of Hematology and Infectious Diseases Coagulopathy in Beta Future Perspectives , 2022 .

[18]  M. Cappellini,et al.  Thalassemia and hypercoagulability. , 2008, Blood reviews.

[19]  M. Cappellini,et al.  Stroke in thalassemia: A dilemma , 2008, American journal of hematology.

[20]  M. Cappellini,et al.  Prevalence of thromboembolic events among 8,860 patients with thalassaemia major and intermedia in the Mediterranean area and Iran , 2006, Thrombosis and Haemostasis.

[21]  E. Cassinerio,et al.  Coagulation and Splenectomy: An Overview , 2005, Annals of the New York Academy of Sciences.

[22]  Winfred C. Wang,et al.  Comparison of transcranial Doppler sonography with and without imaging in the evaluation of children with sickle cell anemia. , 2004, AJR. American journal of roentgenology.

[23]  R. Adams,et al.  Can peak systolic velocities be used for prediction of stroke in sickle cell anemia? , 2004, Pediatric Radiology.

[24]  A. Eldor,et al.  The hypercoagulable state in thalassemia. , 2002, Blood.

[25]  Scott T. Miller,et al.  Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography. , 1998, The New England journal of medicine.

[26]  M A Waclawiw,et al.  Stroke prevention trial in sickle cell anemia. , 1998, Controlled clinical trials.

[27]  P. Lane Sickle cell disease. , 1996, Pediatric clinics of North America.

[28]  R. Adams,et al.  The use of transcranial ultrasonography to predict stroke in sickle cell disease. , 1992, The New England journal of medicine.