Objective: To evaluate clinical characteristics and prognosis of primary myelofibrosis (PMF) patients with thrombocytopenia in varied degrees. Methods: Clinical features and survival data of 1 305 Chinese patients with PMF were retrospectively analyzed. The prognostic value of thrombocytopenia in patients with PMF was evaluated. Results: 320 subjects (47%) presented severe thrombocytopenia (PLT<50×10(9)/L), 198 ones (15.2%) mild thrombocytopenia [PLT (50-99)×10(9)/L] and 787 ones (60.3%) without thrombocytopenia (PLT ≥ 100×10(9)/L). The more severe the thrombocytopenia, the higher the proportions of HGB<100 g/L, WBC<4×10(9)/L, circulating blasts ≥ 3%, abnormal karyotype and unfavourable cytogenetics (P<0.001, P<0.001, P=0.004, P<0.001 and P<0.001, respectively) were observed in this cohort of patients. The more severe the thrombocytopenia, the lower the proportion of JAK2V617F positive (P<0.001) was also noticed. Platelet count was positively correlated with splenomegaly, HGB and WBC (P<0.001, correlation coefficients were 0.131, 0.445 and 0.156, respectively). Platelet count was negative correlated with constitutional symptoms and circulating blasts (P=0.009, P=0.045, respectively; correlation coefficients were -0.096 and -0.056, respectively). The median survival of patients with severe thrombocytopenia, mild thrombocytopenia and without thrombocytopenia were 32, 67 and 89 months, respectively (P<0.001). Multivariate analysis identified thrombocytopenia in varied degrees (HR=1.693, 95%CI 1.320-2.173, P<0.001) and Dynamic Internation Prognostic Scoring System(DIPSS) prognostic model (HR=2.051, 95%CI 1.511-2.784, P<0.001) as independent risk factors for survival. Conclusion: PMF patients with severe thrombocytopenia frequently displayed anemia, leucopenia, circulating blasts and short survival, so active treatment measures should be taken especially in these patients.
Humans ; Primary Myelofibrosis ; Prognosis ; Retrospective Studies ; Thrombocytopenia

Objective To evaluate the clinical safety and efficacy of percutaneous interventional therapy in pediatric patients with secundum atrial septal defect(ASD).Methods Clinical data of 40 patients(age≤2 years)with secundum atrial septal defect treated in our hospital from February 2014 to December 2017 were analyzed retrospectively. There were 13 males and 27 females in these patients.Ultrasound of heart showed that there were 37 patients with single ASD,3 patients with multiple ASDs.One associated with pulmonary stenosis(PS),and 1 associated with patent ductus arteriosus.There were 6 patients with pulmonary hypertension, and the diameter of ASD was (10.6 ± 2.0) mm. All patients were proved to have secundum atrial septal defect before intervention.In the intervention,the transport system was delivered along the femoral vein,inferior vena cava and right atrium through atrial septal defect to the left atrium,and the occluder was released there. Results Of the 40 patients, 38 cases were successfully implanted, and the other two patients were not satisfied with the location of occlusion.The diameter of the ASD occluder was(12.0±2.1)mm and the transport sheath 7-9 F.Plug2 occluder was implanted in the patient with patent ductus arteriosus.To the patient with PS,pulmonary valve balloon angioplasty was performed,and then the pressure gradient reduced obviously, after that ASD occlusion was performed. The total follow up period was from 2 months to 3 years.No residual shunt and unsatisfactory device position were found during the follow up period.The pulmonary pressure reduced to normal,and the right atrium and right ventricle were smaller in a different degree. All patients had no arrhythmia and other complications.Conclusion Transcatheter closure of ASD is safe,reliable,and has fewer complications.It is worthy of popularization and application.Appropriate occluder should be selected according to the size and edge of ASD to reduce complications,such as residual shunt and valve injury.

Ferroptosis is distinct from apoptosis,autophagy,necrosis,cornification and other cell deaths from morphological,biochemical as well as genetic aspects.Ferroptosis plays a critical role in neurological diseases and cancers.Neurological diseases,such as Alzheimer's disease,Parkinson's disease,Huntington's disease,stroke,periventricular leukomalacia and so on,are characterized by multiple etiologies and mechanisms,and are potentially correlated with ferroptosis.Based on the recent researches on ferroptosis and neurological diseases,this review investigates ferroptosis and its role in neurological diseases.

Objective To analyze the peri-procedural complications of coronary rotational atherectomy. Methods A total of 107 consecutive patients with coronary artery disease received rotational atherectomy from January 2012 to December 2016 were enrolled retrospectively. The occurrence rate and the management of peri-procedural complications were summarized. Results ① The serum level of CK-MB and cTnI in patients who received rotational atherectomy were signifi cantly elevated compared with the level before the procedure,[4.1(2.4-14.1)U/L vs.1.5(1.0-2.1)U/L,P<0.001]and[0.28(0.11-1.11)ng/ml vs.0.01 (0.01-0.02) ng/ml, P<0.001], respectively. Of the 107 target lesions, 104 (97.2%) were classifi ed as type B2 or C lesions.②Peri-procedural complications of coronary rotational atherectomy occurred in 21 patients (19.6%). There were 5 cases (4.7%) of coronary slow fl ow, 1 case (0.9%) of severe coronary spasm, 9 cases (8.4%) of coronary dissection after rotational atherectomy and 1 case (0.9%) of side branch occlusion. Six patients (5.6%) had severe sinus bradycardia, but only 1 patient required temporary pacemaker. Burr entrapment happened in 2 patients (1.9%). Peripheral vascular complications occurred in 2 patients (1.9%) with 1 case of femoral artery hematoma at puncture site and the other 1 case of mediastinal hematoma. One patient (0.9%) developed contrast induced nephropathy after the procedure. ③All the peri-procedural complications of rotational atherectomy were benign. The procedure instant success rate was 98.1%, with no acute or sub-acute stent thrombosis, cardiac death, emergent coronary artery bypass graft occurred during hospitalization. Conclusions The complications associated with coronary rotational atherectomy are not rare, but severe complications are not common. The procedure could be safely performed with a high instant success rate.