Hematologic responses to deferasirox therapy in transfusion-dependent patients with myelodysplastic syndromes

Background

Reductions in transfusion requirements/improvements in hematologic parameters have been associated with iron chelation therapy in transfusion-dependent patients, including those with myelodysplastic syndromes; data on there reductions/improvements have been limited to case reports and small studies.

Design and Methods

To explore this observation in a large population of patients, we report a post-hoc analysis evaluating hematologic response to deferasirox in a cohort of iron-overloaded patients with myelodysplastic syndromes enrolled in the Evaluation of Patients’ Iron Chelation with Exjade^® (EPIC) study using International Working Group 2006 criteria.

Results

Two-hundred and forty-seven, 100 and 50 patients without concomitant medication for myelodysplastic syndromes were eligible for analysis of erythroid, platelet and neutrophil responses, respectively. Erythroid, platelet and neutrophil responses were observed in 21.5% (53/247), 13.0% (13/100) and 22.0% (11/50) of the patients after a median of 109, 169 and 226 days, respectively. Median serum ferritin reductions were greater in hematologic responders compared with non-responders at end of study, although these differences were not statistically significant. A reduction in labile plasma iron to less than 0.4 μmol/L was observed from week 12 onwards; this change did not differ between hematologic responders and non-responders.

Conclusions

This analysis suggests that deferasirox treatment for up to 1 year could lead to improvement in hematologic parameters in some patients with myelodysplastic syndromes.     

Safety in the Use of Dexmedetomidine (Precedex) for Deep Brain Stimulation Surgery: Our Experience in 23 Randomized Patients

Objective: Evaluation of safety and efficacy of dexmedetomidine in deep brain stimulation (DBS) surgery. Materials and Methods: A cohort of 23 patients, candidates for DBS for Parkinson's disease, Tourette syndrome, or obsessive‐compulsive disorder, was randomized in two groups: dexmedetomidine group and control group. Standard anesthesiologic parameters were recorded and analyzed, together with the need for other medications. A ten‐degree scale (visual analog scale) assessing patient discomfort during DBS also was recorded at the end of surgery. Results: The results demonstrated good stability of intraoperative monitoring: no respiratory depression and good overall cooperation with the neurologist, while no side‐effects were recorded. Conclusions: Our conclusion is that dexmedetomidine should be considered as a valuable option for sedation in poorly collaborating patients undergoing DBS surgery.     

Sudden unexpected death in epilepsy: From the lab to the clinic setting

Sudden unexpected death in epilepsy (SUDEP) is defined as sudden, unexpected, witnessed or unwitnessed, non-traumatic, and non-drowning death in a patient with epilepsy. Sudden unexpected death in epilepsy is probably the most common cause of epilepsy-related deaths. Many predisposing and initiating factors may coexist and contribute to SUDEP, but the mechanisms are poorly understood. Cardiac and respiratory deregulation seems to have a major role in SUDEP. Here, we review several advances in understanding the mechanisms involved in SUDEP.This article is part of a Special Issue entitled “The Future of Translational Epilepsy Research”.