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 (ß-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.

A comprehensive update of ICET-A Network on COVID-19 in thalassemias: what we know and where we stand.

A review of the literature on COVID-19 pandemic in patients with thalassemias is presented. Globally, the prevalence of COVID-19 among  ß-thalassemia patients seems to be lower than in general population; associated co-morbidities aggravated the severity of  COVID- 19, leading to a poorer prognosis, irrespective of age. A multicenter registry will enhance the understanding of COVID-19 in these patients and will lead to more evidence-based management recommendations.

Preliminary Data on COVID-19 in Patients with Hemoglobinopathies: A Multicentre ICET-A Study.

OBJECTIVES: This study aims to investigate, retrospectively, the epidemiological and clinical characteristics, laboratory results, radiologic findings, and outcomes of COVID-19 in patients with transfusion-dependent ß thalassemia major (TM), ß-thalassemia intermedia (TI) and sickle cell disease (SCD). DESIGN: A total of 17 Centers, from 10 countries, following 9,499 patients with hemoglobinopathies, participated in the survey. MAIN OUTCOME DATA: Clinical, laboratory, and radiologic findings and outcomes of patients with COVID-19 were collected from medical records and summarized. RESULTS: A total of 13 patients, 7 with TM, 3 with TI, and 3 with SCD, with confirmed COVID-19, were identified in 6 Centers from different countries. The overall mean age of patients was 33.7±12.3 years (range:13-66); 9/13 (69.2%) patients were females. Six patients had pneumonia, and 4 needed oxygen therapy. Increased C-reactive protein (6/10), high serum lactate dehydrogenase (LDH; 6/10), and erythrocyte sedimentation rate (ESR; 6/10) were the most common laboratory findings. 6/10 patients had an exacerbation of anemia (2 with SCD). In the majority of patients, the course of COVID-19 was moderate (6/10) and severe in 3/10 patients. A 30-year-old female with TM, developed a critical SARS-CoV-2 infection, followed by death in an Intensive Care Unit. In one Center (Oman), the majority of suspected cases were observed in patients with SCD between the age of 21 and 40 years. A rapid clinical improvement of tachypnea/dyspnea and oxygen saturation was observed, after red blood cell exchange transfusion, in a young girl with SCD and worsening of anemia (Hb level from 9.2 g/dl to 6.1g/dl). CONCLUSIONS: The data presented in this survey permit an early assessment of the clinical characteristics of COVID 19 in different countries. 70% of symptomatic patients with COVID- 19 required hospitalization. The presence of associated co-morbidities can aggravate the severity of COVID- 19, leading to a poorer prognosis irrespective of age.