Background::Although the treatment of peripheral T-cell lymphoma (PTCL) has undergone advancements during the past several years, the response rate and long-term effects with respect to patients with PTCL remain unsatisfactory—particularly for relapsed or refractory (R/R) patients. This phase II trial was designed to explore the efficacy and safety of an all-oral regimen of chidamide plus prednisone, cyclophosphamide, and thalidomide (CPCT) for R/R PTCL patients who could not tolerate the standard chemotherapy for a variety of reasons.Methods::We conducted a multicenter phase II clinical trial in which we combined chidamide (30 mg twice weekly) with prednisone (20 mg daily after breakfast), cyclophosphamide (50 mg daily after lunch), and thalidomide (100 mg daily at bedtime) (the CPCT regimen) for a total of fewer than 12 cycles as an induction-combined treatment period, and then applied chidamide as single-drug maintenance. Forty-five patients were ultimately enrolled from August 2016 to April 2021 with respect to Chinese patients at nine centers. Our primary objective was to assess the overall response rate (ORR) after the treatment with CPCT.Results::Of the 45 enrolled patients, the optimal ORR and complete response (CR)/CR unconfirmed (CRu) were 71.1% (32/45) and 28.9% (13/45), respectively, and after a median follow-up period of 56 months, the median progression-free survival (PFS) and overall survival (OS) were 8.5 months and 17.2 months, respectively. The five-year PFS and OS rates were 21.2% (95% confidence interval [CI], 7.9-34.5%) and 43.8% (95% CI, 28.3-59.3%), respectively. The most common adverse event was neutropenia (20/45, 44.4%), but we observed no treatment-related death.Conclusion::The all-oral CPCT regimen was an effective and safe regimen for R/R PTCL patients who could not tolerate standard chemotherapy for various reasons.Trial Registration::ClinicalTrials.gov, NCT02879526.

Objective:To explore the therapeutic characteristics of population with gout achieving treat-to-target (T2T) indicators through real-world research and evaluate their safety.Methods:A total of 3 287 patients diagnosed with gout by rheumatologists in 21 first-class tertiary hospitals in 10 provinces, municipalities, and autonomous regions in China from January 2015 to December 2021 were included in this polycentric cross-sectional study. The database included patients′ general information, disease characteristics, and clinical application of traditional Chinese and Western medicine treatment measures. SPSS and Excel software were used for data analysis. Frequency analysis, cluster analysis, and factor analysis were used to summarize the characteristics and rules of treatment measures for patients with gout who achieved the target after treatment. The occurrence of adverse events (AE) was recorded during treatment.Results:After treatment, 691 visits (7%) achieved the serum urate (SUA) target, and the most frequent use of urate-lowering therapy (ULT) was febuxostat, followed by benzbromarone. The most common treatment options were following: GroupⅠ: traditional Chinese medicine (TCM) decoction-TCM external treatment-physical exercise-proprietary Chinese medicine; GroupⅡ: ferulic acid-nonsteroidal anti-inflammatory drugs (NSAIDs); Group Ⅲ: allopurinol-sodium bicarbonate-benzbromarone; Group Ⅳ: glucocorticoid-colchicine; Group Ⅴ: febuxostat. A total of 5 898 visits (60%) chieved manifestations of joint pain VAS scores target, and the most frequently used drug to control joint symptoms was NSAIDs. The frequency of use of drugs to control joint symptoms were 2 118 times (usage rate reached 35.9%), while the frequency of ULT were 2 504 times (usage rate reached 42.5%), which was higher than the joint symptom control drug. The most common treatment options were following: Group Ⅰ: proprietary Chinese medicine-TCM decoction-TCM external treatment-physical exercise; Group Ⅱ: NSAIDs-colchicine hormones; Group Ⅲ: allopurinol, Group Ⅳ: benzbromarone; Group Ⅴ: febuxostat. A total of 59 adverse events occurred during treatment.Conclusion:The proportions of gout patients who reach target serum urate level & good control of joint symptoms are both very low, and ULT and anti-inflammatory prescription patterns are very different from international guidelines, so it is necessary to strengthen the standardized management of gout patients. At the same time, life intervention measures account for a certain proportion of the treatment plans for the T2T population, and further exploration is needed.

Objective:To investigate the clinical significances of evaluation indexes of right heart function injury in patients with BCR-ABL-negative myroproliferative neoplasms (MPN).Methods:The clinical data of 208 patients with BCR-ABL-negative MPN diagnosed in the Second People's Hospital of Lianyungang and Jiangsu Province Hospital from January 2015 to August 2021 were retrospectively analyzed, including 63 cases of primary myelopathic fibrosis (PMF), 39 cases of polycytosis vera (PV) and 106 cases of essential thrombocythemia (ET). The clinical characteristics of patients and the examination results of hematological related indicators were compared among the three groups. The examination results of indexes of right heart function injury were analyzed, including echocardiography, brain natriuretic peptide, soluble growth stimulation expression gene-2 (sST-2), lactate dehydrogenase (LDH), D-dimer, ferritin, β 2-microglobulin, peripheral blood WT1 gene, CD34 + cell count, etc. Results:Of the 208 patients, 109 were male and 99 were female; the median age was 62 years old (23 years old, 89 years old). The differences in levels of hemoglobin, platelet count, D-dimer, LDH and ferritin among PMF, PV and ET patients were statistically significant (all P < 0.05). Color echocardiography was performed in 87 patients, including 26 cases of PMF, 19 cases of PV and 42 cases of ET. Pulmonary artery pressure increased in 69 cases (79.3%), left atrial diameter increased in 76 cases (87.3%), and diameter increased during right ventricular diastolic period in 59 cases (67.8%). There were significant differences in pulmonary artery pressure, left atrial diameter and diameter during right ventricular diastolic period among PMF, PV and ET patients (all P < 0.05). Pearson correlation analysis showed that pulmonary artery pressure was positively correlated with ferritin, LDH, sST-2 and age ( r values were 0.796, 0.768, 0.915 and 0.734, all P<0.05), while it was negatively correlated with platelet count ( r = -2.330, P = 0.034). Conclusions:For BCR-ABL-negative MPN patients, the increase of pulmonary artery pressure, ferritin and LDH and the decreased platelet count and hemoglobin may increase the probability of right heart function impairment. For BCR-ABL-negative MPN patients with the higher levels of ferritin, LDH, sST-2, age, and the lower level of platelet count, the pulmonary artery pressure may be higher.

Objective:To investigate the changes of related indicators of right heart hypofunction in patients with primary myelofibrosis (PMF).Methods:The clinical data of 55 PMF patients in the Second People's Hospital of Lianyungang in Jiangsu Province and Jiangsu Province Hospital from January 2015 to August 2019 were retrospectively analyzed. The differences in right heart function-related echocardiographic indexes and biochemical indexes between pre-fibrosis/early stage fibrosis patients and obvious stage fibrosis patients were compared. Single factor linear regression method was used to analyze the correlations of pulmonary artery pressure with biochemical indexes.Results:The hemoglobin level [119 g/L (47-224 g/L) vs. 78 g/L (33-182 g/L)] and platelet count [233×10 12/L (5×10 12/L-984×10 12/L) vs. 117×10 12/L (7×10 12/L-731×10 12/L)] of patients in the pre-fibrosis/early stage fibrosis group were higher than those in the obvious stage fibrosis group, and the differences were statistically significant (both P<0.05). Among 22 patients with complete results of cardiac ultrasound, 90.9% (20/22) patients had increased pulmonary artery pressure, 72.7% (16/22) patients had increased left atrial diameter, and 90.9% (20/22) patients had increased right ventricular diastolic diameter. There were no patients with abnormal ejection fraction. The pulmonary artery pressure [48 mmHg (46-90 mmHg) vs. 33 mmHg (20-50 mmHg) (1 mmHg = 0.133 kPa)], left ventricular diastolic diameter [46 mm (36-50 mm) vs. 47 mm (43-53 mm)] and fractional shortening rate [38.1% (36.0%-38.9%) vs. 35.4% (32.7%-37.8%)] of patients in the pre-fibrosis/early stage fibrosis group were higher than those in the obvious stage fibrosis group, and the differences were statistically significant (all P < 0.05). The pulmonary artery pressure of patients had positive correlations with age ( r = 0.590), serum ferritin (SF) ( r = 0.608), lactate dehydrogenase (LDH) ( r = 0.711) and soluble growth-stimulating expression gene 2 (ST-2) ( r = 0.580)(all P<0.05), and had negative correlation with platelet count ( r = -0.596, P = 0.003). Conclusion:PMF patients are prone to right heart hypofunction, the pulmonary artery pressure is higher in older patients and patients with high SF, LDH and ST-2 levels and low platelet count.

Objective: To investigate the characteristics in pathological diagnosis, clinical features, treatment and prognosis of adult patients with pediatric-type follicular lymphoma (PTFL) . Methods: The clinical and pathological features, laboratory examination, diagnosis and treatment, follow-up results of 5 adult PTFL patients admitted in Jiangsu Province Hospital were retrospectively analyzed, and literature review was conducted in combination with related reports. Results: All 5 patients developed PTFL in their adulthood with a median age of 22 years old (15-33 years) . The initial inanifestation of the disease was local painless lymphadenopathy with no fever, night sweats, emaciation or other systemic B symptoms. Pathological characteristics including typical large follicular structures and high proliferation index were found. Meanwhile, additional clonal rearrangement of immunoglobulin heavy chain gene was observed. However, there was no BCL-2 expression in histochemistry as well as BCL-2 gene abnormality in fluorescence in situ hybridization among these PTFL patients. These adult PTFL patients were all in stage Ⅰ-Ⅱ of the disease. For treatment, they were only treated with local surgical excision after diagnosis while didn’t receive subsequent local radiotherapy or systemic immunochemotherapy. During a median follow-up of 27 months, the 5 cases of PTFL kept in a state of sustained complete remission. Conclusion Adult-onset PTFL is characterized by high pathological proliferation index, while no BCL-2 expression or BCL-2 gene abnormality. Besides, PTFL is clinically manifested as a localized disease that can achieve a quite good prognosis through local surgical intervention. The aforementioned attributes of PTFL are distinctly different from classic adult follicular lymphoma.

Objective To investigate the CT imaging features of basal cell adenoma in the parotid gland and thus to improve the preoperative diagnostic accuracy.Methods The clinical materials and image findings of 8 cases with parotid basal cell tumor,which were proved by pathology,were retrospectively studied.Results All 8 patients had solitary BCA lesion,which involved both the superficial and deep lobe(n =1) and located at the superficial lobe of parotid gland(n =7).All the 6 tumors were clear boundary and round shape without lobular appearance.The diameters of the max lesions ranged from 10.3-27.6 mm.CT scan showed that all lesions were solid nodules with uniform or uneven density.Cystic degeneration was displayed in 5 lesions,among them,cystic area was ≥90％ in 1 lesion.At enhanced scanning phase,most tumors showed a strong enhancement at the arterial phase and a pattern of persistent strong enhancement or slow decline at the venous phase.Conclusion The multi-slice CT imaging features of BCA in parotid gland are characteristic,which is helpful to make qualitative diagnosis in combination with clinical materials.

Marginal zone lymphoma (MZL) accounts for approximately 10 % of non-Hodgkin lymphoma (NHL). It can be divided into three specific entities:extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), nodal marginal zone lymphoma (NMZL) and splenic marginal zone lymphoma (SMZL). MALT lymphoma is the most frequent overall, representing 7.5 % of all NHLs. Reports on research progress of MZL in the 58th American Society of Hematology Annual Meeting covered multiple respects which ranged from basic research to clinical prognosis and treatment. Based on the technology such as flow cytometry, cytogenetics and FISH, further study on pathogenesis of MZL is developing, and new prognostic index system can help to stratify patients more exactly and give a guidance to treatment. What's more, the change of therapy and new drugs will benefit to the clinical efficacy and safety of MZL patients.

Objective The purpose of this study was to establish a rapid method for synthesis and detection of guild RNA (gRNA) which is an essential component in CRISPR/Cas9 knockout technology .Methods First, the Nkp46 gRNA core fragment was synthesized as amplification template .Second , the forward and reversed primers of the matched gRNA were designed using the Nkp46 gene as reference sequence .Third, the DNA fragment of Nkp46 gRNA was amplified by PCR technology using the synthesized gRNA core fragment as template .The gRNA was reversely transcribed in vitro u-sing amplified DNA fragment as template .The efficiency and specificity of gRNA and its interaction with Cas 9 were detec-ted in vitro.Results The specificity and activity of Nkp46 gRNA were high .The obtained gRNA interacted with Cas 9 en-zyme and successfully cut the target double-stranded DNA at the designed site .Conclusions The method for synthesis and detection of gRNA established in this study is faster than the original method , and the created gRNA is fully functional .

Objective To discuss the clinical outcomes of old fracture-dislocation of proximal interphalangeal joint treated with a self-made distraction external fixator which consists of 3 pins and rubbers.Methods From March 2005 to August 2011,8 cases of old fracture-dislocation of proximal interphalangeal joint treated with a pins-rubber distraction external fixator were retrospectively reviewed.There were 6 males and 2 females,aged from 17 to 58 years,averaged 23.2 years.The middle finger was involved in 5 cases and ring finger was involved in 3 cases.The right hand was affected in six patients,and the left hand was affectd in two patients.The mechanism of injury was sports injury in 6 cases,traffic injuries in 1,and sprain in 1.All of the cases were closed injury.The mean time from injury to surgery was 58 d (range,45-90 d).According to Schenck classification of the proximal interphalangeal joint fracture-dislocation,there were 4 cases of type Ⅰ,3 of type Ⅱ,I of type Ⅲ.Based on the Michigan Hand Outcomes Questionnaire (MHQ) for subjective hand function score,the average preoperative score was 56; the range of motion of proximal interphalangeal joint was 0°.Grip strength was 60％-80％ of the contralateral hand.Results The follow-up lasted 6-51 months,with a mean period of 20.5 months.Solid bone healing was eventually achieved in all patients.The average healing period was 3 months (range,2-5 months).The mean normalized MHQ score was 88 (range,84-92).Grip strength was 85％-100％ of the contralateral hand.Extension of proximal interphalangeal joint was -10°-0° (average,-5°).Flexion angle was 75°-100° (average,89.4°).Postoperative complications included slight joint space narrowing (2 cases),joint incongruity (＜ 1 mm) (2 cases) and fusiform arthrocele (2 cases).No infection,pin loosening,and pin breakage was encounted.Conclusion A pins-rubber distraction external fixator is a minimally invasive,reliable and effective treatment for old fracture-dislocation of proximal interphalangeal joint.

Objectives To compare total laparoscopic gastrectomy with intracorporeal hand-sewn Gl reconstruction and laparoscopy-assisted gastrectomy for gastric cancer. Methods Between July 2009 and July 2010, 21 patients of gastric cancer underwent total laparoscopic D2 radical gastrectomy with intracorporeal hand-sewn reconstruction and 28 did laparoscopy-assisted D2 radical gastrectomy in Xijing Hospital of Digestive Diseases. All patients were operated on by an experienced surgeon. Patient demographics, TNM stage, location of tumor, the intraoperative and postoperative details of the two groups were compared. Results In the 21 patients undergoing total laparoscopic gastrectomy, there were 15 of distal gastrectomy and 6 of total gastrectomy, compared with 21 and 7 in laparoscopy-assisted group. In total laparoscopic group, intracorporeal hand-sewn technique was used for gastro-jejunal and jejuno-jejunal (J-J)anastomosis, and 25 mm circular stapler was used for esophago-jejunal anastomosis. The operation time was significant longer in total laparoscopic group than in laparoscopy-assisted group of (279 ± 65 ) min vs.(232 ±40) min (P ＜ 0.05 ). No significant difference was observed between the two groups in proximal margin [(5.7 ± 1.5 )cm vs. (5.1 ± 1.4) cm, P ＞ 0.05] and distal margin [( 3.1 ± 0.9 )cm vs. ( 2.9 ±0.9) cm,P ＞0.05]. The iv narcotic use in laparoscopy-assisted group was 1.8 d but it was not used in total laparoscopic group. The first passing flatus was on day 3 in total laparoscopic group compared with 4.8 d in laparoscopy-assisted group. Both groups had 2 postoperative early complications, one intra-abdominal infection and one lung infection in total laparoscopic group compared with one wound infection and one lung infection in laparoscopy-assisted group. There was no anastomosis-related complications after 4 months of follow-up. Conclusions The operation time and postoperative early complication was acceptable for selected patients treated by total laparoscopic D2 radical gastrectomy with intracorporeal hand-sewn GI tract reconstruction in hands of experienced laparoscopic surgeon.