In this study we provide evidence that the sera of patients with hairy cell leukemia (HCL) contain a factor that can prevent the binding of a monoclonal antibody specific for interleukin-2 receptor (IL-2R) to its target. This factor corresponds to the soluble form of IL-2R (sIL-2R), as assessed by a specific enzyme-linked immunosorbent assay test, and appears to be released by neoplastic hairy cells. The serum sIL-2R levels were very high at diagnosis and significantly reduced during recombinant alpha-interferon (rIFN alpha 2) therapy. Values of sIL-2R appeared to be inversely related to the natural killer in vitro function displayed by peripheral blood mononuclear cells from the same patients. The presence of sIL-2R in the serum of patients with HCL might be involved in the impairment of cell-mediated immunity observed in these patients and could represent a valuable marker for monitoring different phases of the disease and for modulating IFN therapy.

Introduction Cardioinhibitory neurocardiogenic syncope (CNS) is caused by inappropriately trigger-activated cardiac reflex which finally precipitates asystole, sinus bradycardia, or atrioventricular block. In the absence of structural heart disease, the prognosis is excellent, but frequent syncopal episodes can have a significant impact on quality of life. No medical therapy has proved to be really effective and studies concerning pacemaker implantation are often controversial. However, in young individuals, especially those under 40 years of age, implanting a pacemaker may carry more risks than benefits. Thus, alternative techniques such as ablation of the cardiac atrial parasympathetic ganglia (GP) have emerged. Case report A 34-year-old man with frequent episodes of reflex syncope performed a tilt test in March 2022 that diagnosed cardio-inhibitory syncope with 13-second asystole interrupted with external cardiac massage and intravenous atropine. In May 2022 he had an MRI scan showing normal biventricular systolic function and absence of late-gadolinium enhancement and underwent loop recorder implantation. He came to our centre in June 2022 and underwent electrophysiological study and electroanatomical mapping of the right atrium (Carto 3) during general anaesthesia. The anatomical areas of the supero-posterior and infero-posterior GPs of the right atrium were identified by electroanatomical mapping of complex fractionated atrial electrograms (CFAE). Radiofrequency pulses (Thermocool 30 watts) were delivered at these points, resulting in the disappearance of the CFAEs and an increase in basal heart rate. The greatest number of ganglia are allocated on the posterior surface of the right atrium and in particular the supero-posterior GP is the nexus point for vagal input to the GPs before innervating the atria. Therefore, it was decided to localise and ablate exclusively the GPs of the right atrium. The procedure was completed without complications. The patient has not complained of any syncopal episodes since, and the loop has not recorded any significant pauses. Conclusions The anatomic ablation of GP only in the right atrium could represent a feasible method to reduce CNS, to increase the warning symptoms period, and to delay as much as possible pacemaker implant, especially in young patients who have experienced a related physical trauma. This approach significantly decreased the procedural time and risks.

Background After a cryptogenic stroke, long-term monitoring is recommended to start an anticoagulation therapy in patients with at least a documented paroxysm of subclinical atrial fibrillation (AF). Literature is sparse about the recurrence of AF (AF burden) after a cryptogenic stroke, but this might have significant implications in terms of therapeutic strategy. Methods This is a retrospective single-center study of 129 patients who received implantable loop recorders (ILRs), after a cryptogenic stroke, between March 2015 and March 2022. All patients were followed through remote monitoring for at least 6 months. The primary endpoint was AF detection; the secondary endpoints were the AF burden, the earliness (within or after 90 days from the ILR implant) of the first AF episode and if there was an association between these two variables. Results Mean age was 70.3 ± 10.4 years old (67 males, 51.9%); the mean value of left ventricular ejection fraction was 61% ± 5.8. Atrial fibrillation has been detected by ILR in 40.3% of patients (AF= 52 patients, NO AF= 77 patients) and each intracardiac electrogram was visually reviewed by two physicians. Median CHAD2S2-Vasc Score was 5 [4-6]; the median AF burden (assessed in 39 of the 52 patients) was 1.2% [0.1%-14.6%]; among these, 23 patients (59%) had the first episode within 90 days from the ILR implant versus 16 patients (41%) which experienced the first episode later than 90 days. AF burden was significantly higher in the first group (median 3.9% [1.2%-30.9%] vs 0.1% [0.03%-0.75%]; p=0.001). Of note the univariate analysis showed that both detection of the first AF episode within 90 days and echocardiographic findings of atrial disease (atrial dilation or diastolic dysfunction) were significantly associated with AF burden > 1% (about 7 hours for month) (respectively OR 16.5; 95% IC=3.34-81.21, p=0.001 and OR 4.5; 95% IC=1.2-17.5, p=0.03); at the multivariate analysis the significance was confirmed for the earliness of the first AF episode (OR 14.6; 95% IC=2.8-76.75, p=0.002). Conclusion In this small, retrospective study, AF was detected by ILR, after a cryptogenic stroke, in more than one third of patients. AF onset during the first 90 days might be a marker of a high AF burden and might highlight patients who could benefit from a rhythm control strategy of AF. Larger studies and clinical outcomes evaluation of these patients are required to confirm our results.