Case report: Cellular therapy for hydroa vacciniforme-like lymphoproliferative disorder in pediatric common variable immunodeficiency with chronic active Epstein-Barr virus infection.

Hydroa vacciniforme-like lymphoproliferative disorder (HV-LPD) is a cutaneous form of chronic active Epstein-Barrvirus (EBV) infection, which can develop into the extremely rare systemic lymphoma. Patients with Inborn errors of immunity (IEI), such as common variable immunodeficiency (CVID), are at higher risk of developing a severe course of infections especially viral and malignancies than the general population. The aim of the study was to present complex diagnostic and therapeutic management of HV-LPD. The clinical diagnosis was confirmed at the histological and molecular level with next generation sequencing. HV-LPD was diagnosed in a patient with CVID and chronic active Epstein-Barr virus (CAEBV) infection. The patient was refractory to CHOP chemotherapy and immunosuppressive treatment in combination with antiviral drugs (prednisone, bortezomib, gancyclovir). The third-party donor EBV-specific cytotoxic T cells (EBV-CTL, tabelecleucel) were used, which stabilised the disease course. Finally, matched unrelated donor hematopoietic cell transplantation (MUD-HCT) was performed followed by another cycle of EBV-CTL.

The influence of low level laser irradiation on vascular reactivity.

PURPOSE: The mechanism of action of low level laser irradiation on tissues is unclear. Authors of publications present the positive clinical impact of low and medium power laser irradiation on vascular reactivity. The purpose of this study was to analyze the role of vascular endothelium in laser-induced constricted by endothelin-1 and phenylephrine. MATERIALS AND METHODS: Experiments were performed on isolated and perfused rat tail arteries of weighing 250-350g male Wistar rats. Contractility of arteries as a response to endothelin-1 and phenylephrine was measured after exposure to laser stimulation (10, 30 and 110mW). RESULTS: Laser irradiation inhibits vascular smooth muscle contraction induced by endothelin-1 and an alpha-adrenergic receptor agonist, phenylephrine proportionally to the laser power. Concentration-response curves were shifted to the right with significant reduction in maximal response. Laser irradiation at the power of 10mW, 30mW, and 110mW reduced the maximum response of arteries stimulated with phenylephrine sequentially to 88%, 72%, and 52%. Similar findings were observed during stimulation of endothelin-1. Laser irradiation at the power of 10mW, 30mW and 110mW resulted in maximal response respectively reduced to 94%, 62% and 38%. CONCLUSION: Our results strongly suggest that during low level laser irradiation vascular smooth muscle cells reactivity is reduced, this effect is present in arteries with normal endothelium. The mechanism of action of laser biosimulation on tissues is unclear. Authors of publications present the positive clinical impact of low level laser irradiation on vascular reactivity.

High-dose, but not low-dose, aspirin impairs anticontractile effect of ticagrelor following ADP stimulation in rat tail artery smooth muscle cells.

OBJECTIVE: To compare effects of low- versus high-dose aspirin coadministered with ticagrelor on the reactivity of vascular smooth muscle cells (VSMCs). METHODS: Wistar rats were orally administered ticagrelor (10 mg/kg) and/or aspirin (2 or 10 mg/kg) (n = 7 per each of 4 groups) or placebo (n = 9) 12 and 2 hours before experiments. Anticontractile effects of ticagrelor were assessed in perfusion solution containing ticagrelor (1 µM/L). Changes in perfusion pressure proportional to the degree of adenosine diphosphate analogue- (2-MeS-ADP-) and phenylephrine-induced constriction of rat tail arteries were evaluated. RESULTS: Pretreatment with high- but not low-dose aspirin enhanced the reactivity of VSMCs only in endothelium-lined vessels. Suppression of 2-MeS-ADP-induced VSMC contraction by ticagrelor observed in arteries with and without endothelium was maintained in endothelialized arteries pretreated only with low-dose aspirin. For endothelium-denuded vessels and low-dose aspirin we observed a significant reduction of the maximal effect of ticagrelor with no rightward shift of the concentration-response curve for phenylephrine. With high-dose aspirin pretreatment ticagrelor exerted no anticontractile effect. CONCLUSION: High-dose, but not low-dose, aspirin impairs the anticontractile effect of ticagrelor on ADP-induced VSMC contraction in the rat model. Both the clinical significance and detailed underlying mechanism of our findings require further investigation.