Objective:To investigate the efficacy and safety of protein A immunoadsorption (PAIA) combined with rituximab (RTX) in highly sensitized patients who underwent haplo-hematopoietic stem cell transplantation (haplo-HSCT) .Methods:The clinical data of 56 highly sensitized patients treated with PAIA and RTX before haplo-HSCT at the First Affiliated Hospital of Soochow University and Soochow Hopes Hematonosis Hospital between March 2021 and June 2023 were retrospectively analyzed. The number of human leukocyte antigen (HLA) antibody types and the mean fluorescence intensity (MFI), humoral immunity, adverse reactions during adsorption, and survival within 100 days before and after adsorption were measured.Results:After receiving the PAIA treatment, the median MFI of patients containing only HLA Ⅰ antibodies decreased from 7 859 (3 209-12 444) to 3 719 (0-8 275) ( P<0.001), and the median MFI of HLA Ⅰ+Ⅱ antibodies decreased from 5 476 (1 977-12 382) to 3 714 (0-11 074) ( P=0.035). The median MFI of patients with positive anti-donor-specific antibodies decreased from 8 779 (2 697-18 659) to 4 524 (0–15 989) ( P<0.001). The number of HLA-A, B, C, DR, and DQ antibodies in all patients decreased after the PAIA treatment, and the differences were statistically significant (A, B, C, DR: P<0.001, DQ: P<0.01). The humoral immune monitoring before and after the PAIA treatment showed a significant decrease in the number of IgG and complement C3 ( P<0.001 and P=0.002, respectively). Forty-four patients underwent HLA antibody monitoring after transplantation, and the overall MFI and number of antibody types decreased. However, five patients developed new antibodies with low MFI, and nine patients continued to have high MFI. The overall survival, disease-free survival, non-recurrent mortality, and cumulative recurrence rates at 100 days post-transplantation were 83.8%, 80.2%, 16.1%, and 4.5%, respectively. Conclusions:The combination of PAIA and RTX has a certain therapeutic effect and good safety in the desensitization treatment of highly sensitive patients before haplo-HSCT.

Objective:To explore the safety and efficacy of TBF conditioning regimen of thiotepa, fludarabine and busulfan in haploidentical hematopoietic stem cell transplantation (haplo-HSCT) for recipients with hematological malignancies unsuitable for BUCY conditioning regimen (busulfan, cyclophosphamide & mBUCY).Method:A retrospective analysis was conducted for 20 recipients with malignant hematologic diseases receiving TBF conditioning regimen before haplo-HSCT at Soochow Hopes Hematologic Hospital from January 2020 to December 2023. The regimen-related toxicity of TBF was assessed by the Bearman scoring criteria. For comparing the safety and efficacy of TBF conditioning regimen with mBUCY regimen, propensity score matching was performed in a ratio of 1: 2 with disease type, patient age and gender as matching factors.Result:Mild oral mucositis and gastrointestinal reaction were major side-effects without severe cardiac events. Median time to neutrophil and platelet engraftments in TBF group was 11 and 18 days with comparable engraftment in mBUCY group. TBF regimen had a significantly lower incidence of grade Ⅱ-Ⅳ acute graft-versus-host disease (aGVHD) than mBUCY at Day 100 (5% vs 35%, P=0.01). No significant inter-group difference existed in overall survival (68% vs 62%, P=0.98) while 1-year incidence of graft-versus-host disease-free, relapse-free survival (GRFS) improved (63% vs 37%, P=0.06) in TBF group. Conclusion:TBF is a promising conditioning regimen with low toxicity and decent safety for haplo-HSCT. TBF patients tend to have a lower incidence of grade Ⅱ-ⅣaGVHD and better GRFS than mBUCY.

To explore the efficacy and safety of immunoadsorption (IA) in removing de novo donor specific antibody (DSA) after allogeneic hematopoietic stem cell transplantation (HSCT), the relevant clinical data were retrospectively reviewed for one female patient of severe aplastic anemia (SAA). Desensitization treatment with IA after HSCT was offered for removing de novo DSA and ultimately promoting platelet engraftment at First Affiliated Hospital of Soochow University in March 2021.

Objective:To report a case of Kufor-Rakeb syndrome caused by novel ATP13A2 mutation, collect the cases related to ATP13A2 gene mutation published in recent years, summarize the clinical manifestations of the disease, and broaden the clinical diagnostic thinking. Methods:The clinical manifestations of a newly diagnosed patient with Kufor-Rakeb syndrome caused by ATP13A2 gene mutation admitted to Zhongda Hospital, Southeast University on November 26, 2021, were summarized. The related cases of ATP13A2 mutation published from January 2000 to December 2021 were searched through the PubMed and CNKI databases using the keywords "ATP13A2" and "Parkinson′s disease". The onset age, clinical symptoms, family history, genetic testing, and levodopa responsiveness results of the patients were collected. Results:The patient is a 52-year-old female with the main clinical symptoms of static tremor and bradykinesia. Physical examination showed a gear like increase in muscle tension in the right upper limb, involuntary shaking of the right hand and slow movement. She had good responsiveness to levodopa, and the magnetic resonance imaging and susceptibility weighted imaging of the head showed a lack of clear observation of bilateral black matter swallowtail sign. Whole exome sequencing showed that mutations c.3010A>G (p.S1004G) and c.1195+5G>A (splice) were found in the ATP13A2 gene, both of which were not reported. The c.3010A>G (p.S1004G) mutation originated from the mother, and the c.1195+5G>A (splice) mutation originated from the father. In the retrospective literature review, a total of 10 cases were collected, with onset ages ranging from 18 months to 24 years. Among them, 4/10 patients′ parents married close relatives, and the clinical manifestations were mainly motor symptoms of Parkinson′s disease. In addition, 5/10 patients had cognitive dysfunction, and 3/10 patients had mental symptoms. And demonstrations of most patients′ magnetic resonance imaging were normal in the early stage of the disease, and as the disease progressed, some patients′ imaging results showed specific changes, such as whole brain atrophy and changes in the corpus callosum. Meanwhile, 8/10 patients showed good responsiveness to levodopa. Conclusions:Kufor-Rakeb syndrome is a special type of adolescent levodopa responsive Parkinson′s disease caused by ATP13A2 mutation, which is an autosomal recessive disorder. In addition to motor symptoms such as static tremor and bradykinesia, its clinical manifestations may also be accompanied by non motor symptoms such as cognitive and psychiatric disorders. The disease responds well to treatment with levodopa.

Objective:To explore the efficacy of tislelizumab combined with umbilical cord blood transplantation (UCBT) in relapsed/refractory acute myeloid leukemia (R/R AML) patients.Methods:The diagnosis and treatment of 1 patient with R/R AML who received tislelizumab bridging to UCBT after the failure of re-induction treatment in the First Affiliated Hospital of Soochow University in November 2021 was retrospectively analyzed.Results:The 59-year-old male patient with R/R AML achieved a complete remission after initial induction chemotherapy regimen of decitabine and venetoclax, and then additional consolidation therapy regimens of decitabine and middle-dose cytarabine, middle-dose cytarabine and idarubicin were performed. The patient relapsed 16 months later and failed to achieve a second remission after re-induction therapy regimens of cladribine, azacitidine, venetoclax combined with chemotherapy, and homoharringtonine, cytarabine combined with granulocyte colony-stimulating factor. Tislelizumab significantly reduced tumor burden and the patient achieved the complete remission after bridging to UCBT. After transplantation, the patient was given maintenance treatment with azacitidine and he had sustained remission without severe transplant-related complications during 9-month follow-up.Conclusions:The use of tislelizumab bridging UCBT can be a potential therapeutic strategy for R/R AML patients.

【Objective】 To investigate the clinical characteristics and diagnosis and treatment of passenger lymphocyte syndrome (PLS) in patients following allogeneic hematopoietic stem cell transplantation (allo-HSCT). 【Methods】 A total of 489 patients who underwent allo-HSCT in Suzhou Hongci Hematology Hospital were retrospectively enrolled. The clinical process, diagnosis and treatment measures and prognosis of four patients complicated with PLS after transplantation were analyzed. 【Results】 Among the 489 patients, 4 were diagnosed with PLS. The blood types of donor/recipient ABO were all secondary incompatible (The blood type of donors were O and the recipients were A or B). The overall incidence of PLS in allo-HSCT was 0.82%(4/489)and 2.2%(4/179)in transplants with donor/recipient secondary incompatible ABO-blood types. PLS occured in 6-13 days after donor stem cell infusion. Clinical manifestations were dizziness and fatigue, low back pain, jaundice, deepening urine, rapid decrease in hemoglobin on laboratory tests, elevated indirect bilirubin and lactate dehydrogenase, positive urobilinogen, positive direct anti-human globulin test (DAT), and anti-A or anti-B antibodies against recipient red blood cells were detected in plasma. After the treatment of O-type washed red blood cells, methylprednisolone, gamma globulin, rituximab and other treatments, the hemolysis was improved. All patients achieved engraftment of neutrophil and platelet. Red blood cell transfusion was halted in 3 weeks. 【Conclusion】 PLS is a rare complication of allo-HSCT, which mainly occurs in allo-HSCT patients with secondary incompatibility of ABO blood group of donor/recipient. The clinical prognosis is good after properly treatment.

Objective:To investigate the therapeutic effects of different surgical methods on papillary thyroid carcinoma of the isthmus (PTCI) and their effects on parathyroid function and thyroglobulin.Methods:Eighty patients with PTCI who underwent treatment in Zhejiang Xin'an International Hospital from January 2016 to January 2021 were included in this study. They were randomly allocated to undergo ipsilateral lobectomy with removal of the isthmus combined with ipsilateral central neck lymph node dissection (group A, n = 40) or total thyroidectomy combined with ipsilateral central neck lymph node dissection (group B, n = 40). We compared intraoperative and postoperative conditions, complications, and hypoparathyroidism between the two groups. We also compared serum thyroglobulin level measured before and 3 days after surgery between the two groups. Results:Operative time was significantly shorter in group A than in group B [(78.95 ± 13.52) minutes vs. (104.23 ± 27.38) minutes, t = -5.23, P < 0.05]. Intraoperative blood loss was significantly less in group A than in group B [(52.32 ± 6.59) mL vs. (75.41 ± 9.98) mL, t = -12.21, P < 0.05]. There were no significant differences in voice handicap index and reflux symptom index scores between the two groups (both P > 0.05). The incidence of complications was significantly lower in group A than in group B (10.00% vs. 30.00%, χ2 = 5.00, P < 0.05). The incidence of hypoparathyroidism was significantly lower in group A than in group B (7.50% vs. 27.50%, χ2 = 5.54, P < 0.05). At 3 days after surgery, serum thyroglobulin level was significantly lower in group A than in group B [(0.82 ± 0.17) μg/L vs. (1.26 ± 0.23) μg/L, t = -9.73, P < 0.05]. Conclusion:Ipsilateral lobectomy with removal of the isthmus combined with ipsilateral central neck lymph node dissection is more effective on PTCI than total thyroidectomy combined with ipsilateral central neck lymph node dissection. The former has little effect on parathyroid function and can reduce serum thyroglobulin level. The study is highly innovative and scientific.

Objective:To investigate the sedation weaning strategies in critically ill patients with mechanical ventilation in pediatric intensive care unit (PICU) and to explore the effect of different sedative weaning patterns on withdrawal syndrome.Methods:A single-center prospective cohort study was conducted from April 1, 2016 to April 30, 2017. One hundred and twelve patients who required mechanical ventilation and benzodiazepines and (or) opioids for at least 5 consecutive days in PICU of Shanghai Children's Medical Center were enrolled. Twenty patients (17.9%) had an intermittent weaning pattern, defined as a 50% or greater increase in daily benzodiazepine and (or) opioid dose after the start of weaning, and the remaining 92 cases (82.1%) had a steady weaning pattern. The demographic and clinical features, duration and dose of sedative and analgesics, and the incidence of withdrawal syndrome were evaluated. Mann-Whitney U test was used for comparison about clinical features between different weaning pattern groups and children with withdrawal syndrome or not. Logistic regression was used to explore the risk factors of withdrawal syndrome. Results:Among the 112 patients, 46 (41.1%) had withdrawal syndrome. The patients with the intermittent weaning pattern had a high score of pediatric risk of mortality Ⅲ (PRISM-Ⅲ) (10.0 (3.5, 12.0) vs. 6.0 (2.0, 10.0), U=654.50, P=0.043) and were prone to re-intubation (35.0% (7/20) vs. 7.6% (7/92), P=0.003). The patients with withdrawal syndrome had longer duration of sedation (19.5 (16.8, 24.3) vs. 10.0 (7.0, 17.3) days, U=743.50, P<0.01), higher incidence of intermittent weaning pattern (32.6% (15/46) vs. 7.6% (5/66),χ 2=11.58, P=0.001), longer PICU hospitalization (19.0 (15.8, 25.3) vs. 12.0 (8.8, 17.0) days, U=755.00, P<0.01) and higher cost (89 (57,109) vs. 53 (32, 79) thousand yuan, U=804.00, P<0.01). Logistic regression showed that intermittent weaning pattern (odds ratio ( OR) =4.85, 95 % confidence interval ( CI) 1.39-16.91, P=0.013), perioperative period of liver transplantation ( OR=6.97, 95 %CI 1.25-39.04, P=0.027) and a cumulative dose of midazolam ≥ 34.7 mg/kg ( OR=8.12, 95 %CI 3.09-21.37, P<0.01) were risk factors of withdrawal syndrome. Conclusions:Withdrawal syndrome is more likely to occur in children who are intermittently weaned from sedation. Steady weaning strategy may help prevent iatrogenic withdrawal syndrome.

Objective:To explore the multiple mediation effects of the positive and negative affect between emotional intelligence and life satisfaction in military personnel.Methods:Totally 967 male military personnel aged 17 to 34 years were investigated with the Schutte's Emotional Intelligence Scale (SSEIS),the Positive Affect and Negative Affect Scale (PANAS) and Life Satisfaction Index A (LSIA).The relationship among these variables was analyzed by using path analysis and Bootstrap test.Results:Totally 68.7％ military personnel were satisfied with their lives.Path analysis showed that emotional intelligence had indirect effects on life satisfaction through the mediation effects of positive affect and negative affect (x2/df=8.88,GFI =0.98,AGFI =0.94,NFI =0.98,IFI =0.98,TLI =0.95,CFI =0.98,RMSEA =0.09).Bootstrap test showed that the mediational path through positive affect and negative affect with the effect size were 31.5％ and 14.8％,and the total mediational effect size was 46.3％.Conclusion:It suggests that positive affect and negative affect may have mediating effects on the relationship between emotional intelligence and life satisfaction among military personnel.

Objective To investigate the cause and treatment for ischemic ophthalmopathy after cataract phacoemulsification.Methods A total of 3000 patients with cataract underwent phacoemulsification were retrospectively analyzed,and 13 patients with ischemic ophthalmopathy.The ischemic optic neuropathy (ION) were in 8 cases,7 cases occurred in age related cataract,1 cases occurred in the dislocation of lens;The central retinal artery occlusion (CRAO) were occurred in 5 cases,1 cases of age-related cataract,2 cases of congenital cataract,2 cases of traumatic cataract.The patients were diagnosed with fundus,visual field and fundus fluorescein angiography respectively,and then were treated with dilation of blood vessels,nutrition nerves and symptomatic treatment.Results The visual acuity in 6 cases with ION after the treatment were improved in different degrees;The visual acuity in 2 cases with CRAO after treatment were improved,3 cases was not recovered.Conclusion Ischemic ophthalmopathy caused by phacoemulsification cataract surgery may be related to the main vascular disease or optic nerve ischemia and hypoxia during operation,and with early detection and treatment,visual acuity can be improved to some extent.