Unit. Patients were aged 24-85 years (median 68), and were diagnosed as having essential thrombocythemia (ET) (n = 149, 45%), polycythaemia vera (PV)(n = 94, 29%), primary myelofibrosis (PMF)(n = 69, 21%) and unclassifiable MPN (n = 17, 5%). Diagnosis were made according to the 2008 WHO Classification. JAK-2 mutation status was either established or revised in our center, but was unknown in 55 (17%) patients. Data about treatment and cardiovascular and thromboembolic incidents was retrieved from medical records. Results: Forty patients (12%) developed thrombosis after median of 36 (range 1-240) months from the time of diagnosis. Fifteen (5%) patients developed venous thrombosis, twenty-four (7%) arterial, while one (0,3%) patient both type of thrombosis. Regarding the disease type, thrombosis was described in 16 patients with ET (11%), 18 with PV (19%) and 6 with PMF (8%). The risk of thrombosis was the highest in patients with previous thrombotic events, but did not correlate with the type of therapy (cytoreductive and/or antiagreggation) nor the JAK-2 status. Summary/Conclusion: The risk of both arterial and venous thrombosis was found to be increased in patients with MPN even after introducing appropriate treatment. The highest risk of post treatment thrombosis was found in patients with previously diagnosed thrombotic events.
Aim. To assess the efficacy of targeted therapy with ruxolitinib in patients with myelofibrosis in real clinical practice in Russia. To determine the prognostic value of spleen reduction in the early stages of ruxolitinib treatment and its effect on overall survival. Materials & Methods. The present retrospective study was based on the data of 10 centers of Russia. It included 56 myelofibrosis (primary or post-polycythemic and post-throm-bocythemic) patients who received ruxolitinib. The median age of patients was 56 years (range 26-76 years). Most of them (59 %) were considered intermediate-1 risk according Results. By the start of data collection most of patients (79 %) had been treated with ruxolitinib. In no case therapy was withdrawn for the reason of drug toxicity. On ruxolitinib constitutional symptoms were reversed in 70 %, 87 %, and 98 % of patients by months 1, 3 and 6, respectively. In 36 % and 46 % of patients by months 3 and 6, respectively, > 50 % decrease in spleen size was observed. Overall, in 31 % and 27 % of cases the size of the spleen decreased by less than 25 % by months 3 and 6, respectively. The factors affecting the changes in spleen size have not been identified. The probability of overall survival by years 2 and 5 of follow-up was 97 % and almost 70 %, respectively. This parameter was significantly affected by the extent of spleen size reduction by month 3 of follow-up as well as by its initial size. Conclusion. Ruxolitinib shows high efficacy for both decrease of general myelofibrosis symptoms and reduction in spleen size. The extent of spleen reduction is an important prognostic factor. It seems, that in patients with insufficient spleen reduction an increase in drug dose is advisable. If it is not possible, alternative methods of treatment should be sought.
Sir: Endotracheal intubation is one of the most common procedures in anesthesiology. However, 86 % of intubated patients experience some trouble with it [1]. Postintubation tracheoesophageal fistulae (TEF) are infrequent (0.3±3 %) in mechanically ventilated patients. This case report highlights the role of imaging in avoiding it.A 36-year-old man was the victim of a motor vehicle crash, suffering a head injury (Glasgow Coma Score 3, brain hemorrhage), blunt chest trauma (bilateral pneumothorax, lung contusion), and spinal cord injury (paraplegia: T8±T9 level]. Tracheotomy was performed on the 16th day. Because of large amounts of saliva in endotracheal aspirates, a TEF occurred on the 27th day. Tracheal and esophageal endoscopy showed a fistula 4 cm below the vocal cords on the left posterior corner of trachea. On the 44th day an esophageal suture and a strape muscle interposition were performed. Thereafter tracheal damages healed. Considering again the chest radiographs led us to consider the possibility of an overinflated tracheal cuff being involved in this TEF (Fig. 1).The probability of iatrogenic tracheoesophageal damage is high in such cases. However, a traumatic origin could not be ignored because of the degree of trauma. Among reported risk factors for tracheal damage in intubated patients, the main origin of TEF is high cuff pressure [2]. The pathogenesis of TEF can be explained by weakness (more stretched and less vascularized) of the posterior tracheal wall, extension of the head that reverses the pressure difference due to added support of the vertebral column, and nasogastric tube to the posterior tracheal wall [3]. Experimental findings confirm the guidelines for inflating an endotracheal cuff to 25 cmH 2 O or less for sealing the airway and not to 35 cmH 2 O to minimize complications [4]. While high cuff pressures are frequent (about 20 % of mechanically ventilated patients [1]), 55 % require more than 35 cmH 2 O for a good seal of the airway. Using a ªminimal leakº technique leads to excessive pressure in 25 % of cases [4]. Finally, 11 % of airways are not sealed despite more than 82 cmH 2 O [1]. Moreover, peak airway pressure linearly increases cuff pressure [4], but high peak pressure also means substantial air leak around the cuff and therefore more cuff inflation. The necessary volume for occlusion varies with muscle tone and consciousness [3].Even with a high-volume±low-pressure cuff, the design of the cuff does not always fit the shape of the human airway [5]. Moreover, folds of cuff can enhance mucosal lesions. Therefore a cuff pressure survey is not the ªmagic bullet.º Chest radiography helps the physician to recognize the correct positioning of the tube and the cuff volume. Inflated cuff should touch, but not distend, the tracheal wall [2]. If the ratio of the inflated cuff diameter to the tracheal lumen diameter exceeds 150 %, tracheal damage (stenosis, tracheomalacia, or TEF) is likely to result.A pressure survey is important because the ªminimal leakº technique does not eliminate over...
CA 6.0 was used for data analysis. The results were presented as median with 95% confidence interval (CI). Mann-Whitney and Spearman rank tests were applied, the differences considered statistically significant with p < 0.05( * ). Results: 23 (63.9%) PMF patients in the study were JAK2V617F-positive, 8 (22.2%) had mutation in CALR gene. JAK2V617F allele burden data was available in 13 PMF patients with median 8.1% (95%CI 3.4-43.8%). The results of hemostatic parameters in PMF patients and controls are shown in Table 1.
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