Hyperhaemolytic transfusion reaction in two ß-thalassaemia major patients: The role of eculizumab.

WHAT IS KNOWN AND OBJECTIVE: Hyperhaemolytic transfusion reactions are rare life-threatening events predominantly affecting patients with haemoglobinopathies. We report two cases in ß-thalassaemia major patients on chronic transfusion therapy and highlight the role of eculizumab in its management. CASE SUMMARY: Patient 1 presented with intravascular haemolysis on day 7 (D7) post-transfusion and responded to treatment with corticosteroids and intravenous immunoglobulin. However, patient 2 presented with severe symptomatic anaemia (D4 post-transfusion) unresponsive to the aforementioned measures. Eculizumab administration led to resolution of the hyperhaemolysis. WHAT IS NEW AND CONCLUSION: We report the successful management of hyperhaemolysis with eculizumab in a ß-thalassemia major patient.

Prediction of long-term survival in patients with transfusion-dependent hemoglobinopathies: Insights from cardiac imaging and ferritin.

AIMS: The current study evaluated the association of echocardiography, cardiac magnetic resonance (CMR), and ferritin data with 10-year survival in thalassemia patients. METHODS: Demographics, ferritin, echocardiography, and CMR parameters of stable consecutive thalassemia patients were prospectively collected. RESULTS: In total, 75 patients (mean age 37 ± 11 years, 45% male) with thalassemia were included and dichotomized based on their survival status after a median follow-up period of 10.3 [9.6-10.9] years. Older age (HR: 1.071, p = 0.001), ferritin ≥2000 ng/ml (HR: 4.682, p = 0.007) and ≥1700 ng/ml (HR: 7.817, p = 0.002), elevated LV end-diastolic pressure (HR: 1.019, p = 0.044), TR Vmax >2.8 m/s (HR: 6.845, p = 0.005), and CMR T2∗ ≤20 msec (HR: 3.602, p = 0.043) and ≤34 msec (HR: 5.854, p = 0.026) were associated with increased all-cause mortality (primary endpoint). A baseline model including age was created and became more predictive of worse survival by adding TR Vmax >2.8 m/s instead of elevated LV end-diastolic pressure (C index 0.767 vs. 0.760, respectively), ferritin ≥1700 ng/ml instead of ≥2000 ng/ml (C index 0.890 vs. 0.807, respectively), or CMR T2∗≤34 msec instead of ≤20 msec (C index 0.845 vs. 0.839, respectively). Parameters associated with the combined endpoint of cardiac mortality/cardiac hospitalization (secondary endpoint) after adjusting for age were ferritin ≥1700 ng/ml (HR 3.770, p = 0.014), ratio E/A wave >2 (HR 3.565, p = 0.04), TR Vmax >2.8 m/s (HR 4.541, p = 0.049), CMR T2∗ ≤20 ms (HR 9.462, p = 0.001), and CMR T2∗ ≤34 ms (HR 11.735, p = 0.002). The model including age and T2∗ ≤34 ms instead of T2∗ ≤20 ms was more predictive of the secondary endpoint (C-index 0.844 vs 0.838, respectively). CONCLUSIONS: In thalassemia patients, TR Vmax >2.8 m/s, ferritin ≥2000 ng/ml, and CMR T2∗ ≤20 ms were associated with worse long-term survival. In the current era of advanced chelation therapy, aiming for ferritin ≤1700 ng/ml and CMR T2∗ ≥34 ms may improve their prognosis.

Left Atrial Strain Identifies Increased Atrial Ectopy in Patients with Beta-Thalassemia Major.

Patients with beta-thalassemia major (ß-ΤΜ) may develop cardiac arrhythmias through a multifactorial mechanism. The current study evaluated the association of cardiac structure and function on echocardiography with atrial ectopic burden on 24-hour tape recording in ß-ΤΜ patients. This prospective study included consecutive ß-ΤΜ patients. Demographic, laboratory, echocardiographic, cardiac magnetic resonance (CMR) T2* and 24-hour tape recording data were prospectively collected. The patients were classified according to the median value of premature atrial contractions (PACs) on 24-hour tape. In total, 50 ß-TM patients (37.6 ± 9.1 years old, 50% male) were divided in 2 groups; PACs ≤ 24/day and > 24/day. Patients with PACs > 24/day were treated with blood transfusion for a longer period of time (39.0 ± 8.6 vs. 32.0 ± 8.9 years, p < 0.007), compared to their counterparts. Older age (OR: 1.121, 95% CI: 1.032-1.217, p = 0.007), longer duration of blood transfusion (OR:1.101, 95% CI:1.019-1.188, p = 0.014), larger LV end-diastolic diameter (OR: 4.522, 95% CI:1.009-20.280, p = 0.049), higher values of LA peak systolic strain (OR: 0.869, 95% CI: 0.783-0.964, p = 0.008), higher MV E/E' average (OR: 1.407, 95% CI: 1.028-1.926, p = 0.033) and higher right ventricular systolic pressure (OR: 1.147, 95% CI: 1.039-1.266, p = 0.006) were univariably associated with PACs > 24/day. LA peak systolic strain remained significantly associated with PACs > 24/day after adjusting for the duration of blood transfusions or for CMR T2*. The multivariable model including blood transfusion duration and LA peak systolic strain was the most closely associated with PACs > 24/day. Receiver operating characteristic curve analysis identified a left atrial peak systolic strain of 31.5%, as the best cut-off value (83% sensitivity, 68% specificity) for prediction of PACs > 24/day. In ß-TM patients, LA peak systolic strain was associated with the atrial arrhythmia burden independently to the duration of blood transfusions and CMR T2*.

A survey to evaluate the implementation of vaccine recommendations among rheumatologists practicing in Greece.

OBJECTIVES: We sought to document the knowledge, perceptions and attitudes toward vaccinations among rheumatologists practicing in Greece. METHODS: Rheumatologists practicing in Greece in 2015 were surveyed by questionnaire during the Postgraduate Course of the Greek Rheumatology Society. Statistical analysis was carried out using SPSS software. RESULTS: One hundred and ten practicing rheumatologists in Greece were surveyed. Response rate was 63%. The majority of responders (82%) inquire patients about vaccination status during rheumatology medical history and most of them specifically advise patients for vaccination uptake (91%). Correct identification of all vaccine types was made by 11% of rheumatologists that took the survey. Ninety-three percent of responders were aware that influenza vaccine should be administered annually, and 94% acknowledged the need for pneumococcal vaccination. Some were not concerned about reduced immunogenicity in patients receiving treatment with methotrexate/TNF inhibitors and rituximab/abatacept (17% and 7%, respectively). A notable percentage overlook that live vaccines are contraindicated during treatment with TNF inhibitors (17%), conventional synthetic DMARDs (61%), or corticosteroids (30%). CONCLUSIONS: According to our results, the majority of Greek rheumatologists have implemented a vaccine strategy in their everyday practice. Still, there are several misconceptions that need to be addressed. A significant percentage cannot properly distinguish between inactivated and live vaccines, and many are not knowledgeable of the potential effect of specific DMARDs on the immunogenicity and safety of vaccination.

A meta-analysis to determine the effect of pharmacological and non-pharmacological treatments on fibromyalgia symptoms comprising OMERACT-10 response criteria.

Fibromyalgia is characterized by widespread pain, sleep problems, fatigue, functional impairment, psychological distress, and cognitive dysfunction. The objective of this meta-analysis is to synthesize the available data on the effectiveness of pharmacological and non-pharmacological interventions across all domains included in the Outcome Measures in Rheumatology Clinical Trials (OMERACT-10) fibromyalgia response definitions, and to examine response based on these definitions. We searched Cochrane, PubMed, Scopus, and the reference lists of articles for randomized controlled trials of any drug formulation or non-pharmacological intervention used for fibromyalgia treatment. We extracted efficacy data regarding pain, sleep, physical function, fatigue, anxiety, depression, and cognition. The available data were insufficient to draw definite conclusions regarding response. Indirect evidence indicates that it may be expected with the use of serotonin noradrenaline reuptake inhibitors (SNRIs), noradrenaline reuptake inhibitors (NRIs), and multidisciplinary treatment.

Role of vaccinations and prophylaxis in rheumatic diseases.

Targeted strategies for reducing the increased risk of infection in patients with autoimmune rheumatic diseases include vaccinations as well as antibiotic prophylaxis in selected patients. However, there are still issues under debate: Is vaccination in patients with rheumatic diseases immunogenic? Is it safe? What is the impact of immunosuppressive drugs on vaccine immunogenicity and safety? Does vaccination cause disease flares? In which cases is prophylaxis against Pneumocystis jirovecii required? This review addresses these important questions to which clinicians and researchers still do not have definite answers. The first part includes immunization recommendations and reviews current data on vaccine efficacy and safety in patients with rheumatic diseases. The second part discusses prophylaxis for Pneumocystis pneumonia.

Comparison of national clinical practice guidelines and recommendations on vaccination of adult patients with autoimmune rheumatic diseases.

The aim of the study is to identify and compare national recommendations on vaccination of adult patients with autoimmune rheumatic diseases (ARDs) in Europe, North America, and Australia. We conducted a search for recommended immunizations in adult patients with ARDs in the Medline database and the Web sites of National Rheumatologic Societies, Ministries of Health, National Advisory Committees on Immunization, and other relevant National Scientific Societies. We compared national guidelines and identified points of agreement and differences. Guidelines on vaccination of adult patients with ARDs were identified in 21 countries. Points of agreement include administering influenza and pneumococcal vaccines in addition to inactivated age-appropriate or travel-related vaccines, and avoiding the use of live vaccines in immunocompromised patients with ARDs. The most important differences concern the steroid dose that induces immunosuppression, the time interval between live vaccines and the initiation of immunosuppressive treatment, herpes zoster vaccination, and the preferred pneumococcal vaccine in patients with ARDs. We observed significant differences among national recommendations on immunizations in patients with ARDs, reflecting the lack of evidence-based data.

Monte Carlo estimation of radiation doses in red bone marrow and breast in common pediatric x-ray examinations.

Radiation exposure was investigated for children undergoing various common radiographies in three dedicated pediatric hospitals in Greece. Kerma in air at the entrance of the beam (Ka,e) was measured with thermoluminescent dosimeters. Ka,e values ranged from 0.09 mGy to 5.52 mGy and were found to be greater in Hospital C, because of the increased high voltage and time-current product used by the radiation technologists. Equivalent doses in red bone marrow and breast were estimated with Monte Carlo simulation by PCXMC code. Values ranged from 2 microSv to 204 microSv for red bone marrow and from 0 to 817 muSv for breast. Variation in doses occurred due to field size, high voltage setting, and Ka,e.

Derivation of ozone flux-yield relationships for lettuce: a key horticultural crop.

Ozone flux-response relationships were derived for lettuce, employing a multiplicative approach to model the manner in which stomatal conductance is influenced by key environmental variables, using a dataset collected during field experimentation in Crete and yield-response relationships derived from parallel open-top chamber experiments. Regional agronomic practices were adopted throughout. Computed versus measured data revealed that the derived model explained 51% (P<0.001) of the observed variation in stomatal conductance. Concentration-based indices were compared with flux-based indices. Analyses revealed a significant relationship between accumulated stomatal ozone flux and yield employing flux threshold cut-offs up to 4 nmol m(-2) s(-1). Regressions employing very low or zero flux thresholds resulted in the strongest yield-flux relationships (explaining approximately 80% (P<0.05) of the variation in the dataset).