Introduction: Hypereosinophilic syndrome (HES) is a very heterogeneous group ofdisorders with varied etiologies characterized by peripheral eosinophilia and eosinophilic tissue/end-organ damage. Three-dimensional speckle-tracking echocardiography (3DSTE) was used for assessment of left ventricular (LV) rotational mechanics in HES patients. Methods:The study comprised 13 HES patients, from which one patient was excluded due to insufficient image quality. The remaining patient population consisted of 12 HES cases (mean age: 59.7 ± 13.7 years, eight males). The control group consisted of 36 healthy volunteers (mean age: 52.9 ± 8.3 years, 23 males). 3DSTE was used for the evaluation of LV rotational abnormalities.Results: Both LV apical rotation (4.86 ± 1.92 degree vs 10.07 ± 3.92 degree, P < .0001) and LV twist (8.52 ± 2.79 degree vs 14.41 ± 4.26 degree, P < .0001) showed significant deteriorations in most of HES patients. Time-to-peak LV apical rotation (380 ± 115 ms vs 344 ± 69 ms, P = .56), LV basal rotation (335 ± 148 ms vs 337 ± 111 ms, P = .89), and LV twist (348 ± 91 ms vs 320 ± 60 ms, P = .64) were not significantly different between HES patients and controls. No correlations could be detected between absolute eosinophil count and eosinophil ratio and apical LV rotation (r = 0.12, P = .51 and r = 0.23, P = .45, respectively) and LV twist (r = 0.24, P = .39 and r = 0.31, P = .34, respectively). In two subjects, the absence of LV twist called LV "rigid body rotation" (RBR) was detected. Conclusions: Reduced LV apical rotation and twist could be demonstrated in HES.LV-RBR could be detected in some HES patients. K E Y W O R D S echocardiography, hypereosinophilic syndrome, left ventricular, rotation, speckle-tracking, three-dimensional, twist | 2065 NEMES Et al.

No abstract

In line with previous studies with originator rhG-CSF, the findings of this study indicate that biosimilar rhG-CSF following AHSCT is effective and generally well tolerated in the engraftment setting. In addition, biosimilar rhG-CSF is comparable to the originator rhG-CSF in terms of kinetics of PBSC mobilization and yield of CD34+ cells. In conclusion, the authors have demonstrated that the use of biosimilar rhG-CSF is effective and safe in autologous PBSC mobilization and engraftment after AHSCT.

Increased LA volumes can be demonstrated in HES patients, accompanied by reduced LA peak circumferential strain as assessed by 3DSTE, suggesting LA remodeling.

Thrombo-haemorrhagic events are the main cause of morbidity and mortality in essential thrombocythemia. The aim of this study was to estimate the incidence of thrombotic events and the impact of the JAK2V617F, MPL (W515L, W515K, W515R, W515A and S505N) and CALR (type-1, type-2) mutations on 101 essential thrombocythaemia patients (72 females and 29 males with a mean age of 61 years) diagnosed in a Southern Hungarian regional academic centre. The incidence of major thrombosis was 13.86 %. Sixty percent of the patients carried the JAK2V617F mutation. The MPL mutations were analysed by sequencing and the W515L was the only one we could identify with an incidence of 3.96 %. Type-2 CALR mutation could be identified in 3 cases among the patients who had JAK2/MPL-unmutated ET. Statistical analyses revealed that the JAK2V617F mutation was associated with significantly increased levels of platelet (p = 0.042), haemoglobin (p = 0.000), red blood cell (p = 0.000) and haematocrit (p = 0.000) and hepatomegaly (p = 0.045) at diagnosis compared to JAK2V617F negative counterparts, however there was no significant association between the JAK2V617F mutation status (relative risk: 1.297, 95 % CI 0.395-4.258; p = 0.668) and subsequent thrombotic complications. The impact of JAK2V617F, MPL W515L and CALR mutations on the clinical findings at the diagnosis of ET was obvious, but their statistically significant role in the prediction of thrombotic events could not be proven in this study. Our results indirectly support the concept that, besides the quantitative and qualitative changes in the platelets, the mechanisms leading to thrombosis are more complex and multifactorial.