Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematopoietic stem cell disease caused by abnormal expression of glycosylphosphatidylinositol (GPI) on the cell membrane due to mutations in the phosphatidylinositol glycan class A(PIGA) gene. It is commonly characterized by intravascular hemolysis, repeated thrombosis, and bone marrow failure, as well as multiple systemic involvement symptoms such as renal dysfunction, pulmonary hypertension, swallowing difficulties, chest pain, abdominal pain, and erectile dysfunction. Due to the rarity of PNH and its strong heterogeneity in clinical manifestations, multidisciplinary collaboration is often required for diagnosis and treatment. Peking Union Medical College Hospital, relying on the rare disease diagnosis and treatment platform, has invited multidisciplinary clinical experts to form a unified opinion on the diagnosis and treatment of PNH, and formulated the Expert Consensus of Multidisciplinary Diagnosis and Treatment for Paroxysmal Nocturnal Hemoglobinuria (2024), with the hope of promoting the standardization of PNH diagnosis and treatment.

Objective:To explore the difference in the complexity of different treatment planning systems, multi-leaf collimator (MLC) types and treatment sites of volume-modulated arc therapy (VMAT), and propose a complexity score for plan quality control.Methods:Statistical analysis of 12 complexity metrics including Monaco and Eclipse, Agility, Millennium and High-definition MLC, nasopharyngeal, lung and cervical cancer was performed. Spearman correlation coefficient between complexity metrics was calculated. Principal component analysis was conducted to reduce the dimensionality of the original data set to the first two principal components and explain its physical meaning. Complexity score based on the principal components was calculated to establish warning and action thresholds for plan quality control. The correlation between complexity metrics and γ pass rate was analyzed.Results:Except cervical cancer aperture sub-regions metric, other metrics had significant differences between Monaco and Eclipse. Monaco MLC had a more regular field but higher MU, smaller leaf gap, and longer leaf travel distance. High-definition MLC with smaller leaf width significantly added MLC aperture-related metrics. The first two principal components explained over 80% of the total variance of the original dataset, complexity score was weighted average of first two principal components. The distribution of complexity score for different equipment and sites was different. The warning threshold was expressed as the average plus standard deviation, and the action threshold was expressed as the average plus 2 standard deviations. Complexity metrics and complexity scores had small correlation with γ pass rate, showing weak or irrelevant but statistically significant. Conclusions:Different planning systems, MLC types, and treatment site complexity metrics are significantly different. The complexity score is a useful tool for plan quality control.

Cutaneous T-cell lymphomas are a relatively rare group of mature T-cell lymphomas mainly manifesting in the skin, and its common subtype is mycosis fungoides. Total skin electron irradiation is one of the important conventional treatment methods, but there are many disadvantages, such as uneven dose distribution, poor position repetition, and long treatment time, which affect the clinical efficacy and patient prognosis. With the emergence and gradual popularization of helical tomotherapy in recent years, more and more medical institutions are gradually expanding their applications in total skin irradiation due to their ability to treat ultra-long targets and achieve dose-sculpted distribution, aiming to further explore its good or bad, and confirm whether it can replace the traditional total skin electron irradiation. In this article, research progress on total skin irradiation using helical tomotherapy was reviewed, the development of treatment technology, clinical efficacy and current concerns and controversies were illustrated.

Objective:To study the changing characteristics and impact factors of helical tomotherapy (HT)for longitudinal dose fall-off outside the target, in order to guide the plan junction or pretreatment target and implementation efficiency in clinical.Methods:Eight patients with head and neck tumors admitted to the Department of Oncology Radiotherapy of the First Affiliated Hospital of Zhengzhou University in December 2019 were retrospectively selected as the research objects. The planning target area and dose drop structure were outlined in the head and neck images with a thickness of 1 mm obtained by Siemens SOMATOM Definition AS positioning computerized tomography (CT). Different field widths (FW, 5.0 cm/2.5 cm/1.0 cm) and pitches (0.430/0.287/0.215) were assembled for planning with the same modulation factor (1.8), finest does calculation grid (0.195 cm ×0.195 cm) and other planning parameters were consistent. The plans were designed by different parameters, and the result was analyzed by univariate analysis.Results:The that different pitch curves coincided under the same field width by comparative analyzing, so pitchs had no effect on dose drop. The different field width curves were independent of each other, indicating that the field width had an effect on dose drop in the head and foot direction. The relationship between the longitudinal dose drop speed outside the target and the change of the field width was inversely correlated: the larger field widths meant the slower dose fall-off and the larger penumbra, while the smaller field widths meant the faster fall-off and the smaller penumbra. When the dose fall-off to 50% of the prescribed dose, the distance from the target was approximately equal to half the field widths, and the pitchs had not affect the rate of dose-drop, while the dose at different distances from the target boundary could be calculated by the fitting formulas. The field widths and pitchs had little effect on the CI and HI index of the target, relatively, the target area was best when the field width was 2.5 cm. The total beam-on time gradually decreased with the increase of the field widths and pitches.Conclusions:When segment target therapy needs to consider planning junction, execution efficiency, and controlling longitudinal dose fall-off and considered the execution, the optimal planned parameters such as field widths and pitches could be selected or the target at the junction regions could be adducted according to the longitudinal dose drop formula, so as to achieve the ideal dose distribution.

Objective:To evaluate the multi-leaf collimator (MLC) performance of TrueBeam accelerator using trajectory log files.Methods:All tests were performed 5 times under different gantry-collimator angle combination. The 1 mm picket fences were constructed by static or dynamic MLC. The control ability for small-field accuracy of accelerator was evaluated. Repeatability was assessed by MLC repeat motion. The movement performance of difference velocities along one direction and the opposite direction were evaluated via a 1 cm picket fences which slipped from -7 cm to 7 cm with a uniform velocity and stopped or immediately back at 7 cm. The MLC performance in a complex program was evaluated by a cross movement test.Results:Both the static and the dynamic picket fences yielded high accuracy. The deviation spectrums of MLC in different gantry angle were consistent, however, an absolute difference of 0.001 1 mm was found. For uniform velocity movement tests with 0°gantry, the RMSE of MLC was increased from 0.015 0 mm to 0.059 8 mm when the speed was accelerated from 5 mm/s to 25 mm/s. Similar results were obtained in non-zero gantry angle. The "overspeed" effect caused by the direction change movement of MLC was less obvious than that caused by speed changed from zero to a uniform velocity movement state. There was no significant change in speed before and after the MLC crossing. The MLC speed fluctuated around the set value, which was independent of the gantry angle.Conclusion:A method for evaluating the performance of MLC using trajectory log files is established, which can evaluate the MLC performance of TrueBeam accelerator and be used for MLC rapid quality control in clinical practice.

Objective:To investigate the features, changing trend, and rules of pneumoconiosis in Jiangsu Province, China, as well as the health status of patients with pneumoconiosis.Methods:From July to October 2019, the patients with pneumoconiosis, reported up to the end of 2018 in Jiangsu Province, were enrolled as respondents, and follow-up and retrospective investigation were performed. A total of 24405 patients with pneumoconiosis were investigated, and related data were collected from the monitoring system of cause of death for residents, pneumoconiosis network reporting system, occupational disease diagnosis institution, management institutions for the reporting of occupational diseases, and related residents' committee or village committee. The patients with pneumoconiosis, who had been reported, were followed up by telephone or on-site visit to obtain the information on their conditions. A descriptive analysis was performed for age of onset, working years, sex, category of industry, type of pneumoconiosis, annual disease onset, geographic distribution, and medical security.Results:Among the 24405 patients, a male/female ratio was 16.81∶1. Of all 24405 patients, 15948 (65.35%) had stage 1 pneumoconiosis, 5289 (21.67%) had stage 2 pneumoconiosis, and 1637 (6.71%) had stage 3 pneumoconiosis. The mean working years for dust exposure was 16.25±9.95 years for all patients, and the mean working years for dust exposure was 15.80±9.95 years for patients with stage 1 pneumoconiosis, 17.82±9.80 years for patients with stage 2 pneumoconiosis, and 16.31±9.90 years for patients with stage 3 pneumoconiosis. The highest number of cases of pneumoconiosis was reported in Wuxi (5744 cases, accounting for 23.54%) , followed by Zhenjiang (4160 cases, accounting for 17.05%) , Xuzhou (3851 cases, accounting for 15.78%) , Yancheng (3340 cases, accounting for 13.69%) , and Suzhou (2948 cases, accounting for 12.08%) . Major types of pneumoconiosis included silicosis (15392 cases, accounting for 63.07%) and coal workers' pneumoconiosis (5253 cases, accounting for 21.52%) . In this survey, 21115 completed follow-up, among whom 15924 survived and 5191 died, 15924 patients with pneumoconiosis survived, among whom 7461 (46.85%) had an age of ≥70 years and 2515 (15.79%) were exposed to dust for 5-9 years. The industries involved were mainly coal mining and washing industry (5687 cases, accounting for 35.71%) and public management, social security, and social organization (3349 cases, accounting for 21.03%) ; in terms of security, 7999 patients (50.23%) were covered by occupational injury insurance, 946 (5.94%) were compensated by employers, 4537 (28.49%) were covered by basic medical insurance for urban and rural residents, 1590 (9.98%) were covered by critical illness insurance, and 5458 (34.28%) were covered by other types of social security, such as medical assistance and poverty relief.Conclusion:Silicosis and coal worker's pneumoconiosis are the key points for the prevention and treatment of pneumoconiosis in Jiangsu Province, and supervision should be strengthened for industries and regions with serious dust hazards.

Objective:To investigate the features, changing trend, and rules of pneumoconiosis in Jiangsu Province, China, as well as the health status of patients with pneumoconiosis.Methods:From July to October 2019, the patients with pneumoconiosis, reported up to the end of 2018 in Jiangsu Province, were enrolled as respondents, and follow-up and retrospective investigation were performed. A total of 24405 patients with pneumoconiosis were investigated, and related data were collected from the monitoring system of cause of death for residents, pneumoconiosis network reporting system, occupational disease diagnosis institution, management institutions for the reporting of occupational diseases, and related residents' committee or village committee. The patients with pneumoconiosis, who had been reported, were followed up by telephone or on-site visit to obtain the information on their conditions. A descriptive analysis was performed for age of onset, working years, sex, category of industry, type of pneumoconiosis, annual disease onset, geographic distribution, and medical security.Results:Among the 24405 patients, a male/female ratio was 16.81∶1. Of all 24405 patients, 15948 (65.35%) had stage 1 pneumoconiosis, 5289 (21.67%) had stage 2 pneumoconiosis, and 1637 (6.71%) had stage 3 pneumoconiosis. The mean working years for dust exposure was 16.25±9.95 years for all patients, and the mean working years for dust exposure was 15.80±9.95 years for patients with stage 1 pneumoconiosis, 17.82±9.80 years for patients with stage 2 pneumoconiosis, and 16.31±9.90 years for patients with stage 3 pneumoconiosis. The highest number of cases of pneumoconiosis was reported in Wuxi (5744 cases, accounting for 23.54%) , followed by Zhenjiang (4160 cases, accounting for 17.05%) , Xuzhou (3851 cases, accounting for 15.78%) , Yancheng (3340 cases, accounting for 13.69%) , and Suzhou (2948 cases, accounting for 12.08%) . Major types of pneumoconiosis included silicosis (15392 cases, accounting for 63.07%) and coal workers' pneumoconiosis (5253 cases, accounting for 21.52%) . In this survey, 21115 completed follow-up, among whom 15924 survived and 5191 died, 15924 patients with pneumoconiosis survived, among whom 7461 (46.85%) had an age of ≥70 years and 2515 (15.79%) were exposed to dust for 5-9 years. The industries involved were mainly coal mining and washing industry (5687 cases, accounting for 35.71%) and public management, social security, and social organization (3349 cases, accounting for 21.03%) ; in terms of security, 7999 patients (50.23%) were covered by occupational injury insurance, 946 (5.94%) were compensated by employers, 4537 (28.49%) were covered by basic medical insurance for urban and rural residents, 1590 (9.98%) were covered by critical illness insurance, and 5458 (34.28%) were covered by other types of social security, such as medical assistance and poverty relief.Conclusion:Silicosis and coal worker's pneumoconiosis are the key points for the prevention and treatment of pneumoconiosis in Jiangsu Province, and supervision should be strengthened for industries and regions with serious dust hazards.

Objective To investigate the optimal distance between upper and lower target volumes and their correlated planning parameters by analyzing the dose distribution in the abutment regions during total body irradiation ( TBI) using helical tomotherapy. Methods A total of 10 patients with acute leukemia and with a height around 120 cm were enrolled. All patients were scanned by a Siemens simulation computerized tomography (CT) at a slice thickness of 5 mm. A lead wire was placed 10. 0 cm above the patella as a marker of the separation boundary for the upper and lower target volumes. The delineations of target volumes and organs at risk ( OARs ) were performed in the Varian Eclipse 13. 5 workstation with targets shrunk beyond the separation boundary at different distances. After contours and CT images were transferred to HT workstation, treatment plans were designed with different field width (FW, 5. 0 cm/2. 5 cm/1. 0 cm) and pitch values (0. 430/0. 287) at a modulation factor of 1. 8. All the plans were optimized with a dose calculation grid of 0. 195 cm × 0. 195 cm and identical planning parameters. The correlation between treatment planning parameters and targets shrunk distances were investigated by analyzing the dose distributions in the abutment area. Results The study demonstrated that the dose distributions in the abutment area were influenced only by the field width parameters: when the gap distance between the upper and lower targets was 5. 0 cm, the optimal FW is 5. 0 cm;Similarly when the gap distances were 2. 0 cm and 1. 0 cm, and the optimal FW 2. 5 cm and 1. 0 cm, respectively. In another words, the dose distribution of the abutment region was optimal when the target gap distance was equal to FW. Pitch values did not affect the quality of dose distribution in the abutment region and the overall treatment time ratio. Overall treatment time was inversely related to the FW. Conclusions Consistent target distance and FW is helpful to improve the dose homogeneity in the abutment area during TBI with HT. Appropriate planning parameters is critical to balance the treatment efficacy and efficiency.

Myeloid differentiation factor 88 (MYD88) is a key linker in the Toll-like receptor (TLR) signaling pathway, which plays an important role in the progression of the tumour. Recent studies have shown that the activating mutation of MYD88 L265P has been identified in about of 90% lymphoplasmacytic lymphoma/Waldenstrom's macroglobulinemia and about of 40% diffuse large B-cell lymphoma and other subtypes of B-cell proliferative neoplasms. Different types of B-cell proliferative neoplasms have their own histology, immunohistochemistry and clinical characteristics, thus, mutation rates of MYD88 L265P are different. This review discusses the latest progress of MYD88 L265P mutation in B-cell proliferative neoplasms.

Objective: To investigate the efficacy and safety of the new investigational drug pegylated interferon α-2b (Peg-IFN-α-2b) (Y shape, 40 kD) injection (180 µg/week) combined with ribavirin in the treatment of patients with genotype 1/6 chronic hepatitis C (CHC), with standard-dose Peg-IFN-α-2a combined with ribavirin as a positive control. Methods: A multicenter, randomized, open-label, and positive-controlled phase III clinical trial was performed. Eligible patients with genotype 1/6 CHC were screened out and randomly divided into Peg-IFN-α-2b(Y shape, 40kD) group and Peg-IFN-α-2a group at a ratio of 2:1. The patients in both groups were given oral ribavirin for 48 weeks in addition and then followed up for 24 weeks after drug withdrawal. Abbott Real Time HCV Genotype II was used to determine HCV genotype, and Cobas TaqMan quantitative real-time PCR was used to measure HCV RNA level at 0, 4, 12, 24, 48, and 72 weeks. Adverse events were recorded in detail. The primary efficacy endpoint was sustained virological response (SVR), and a non-inferiority test was also performed. Results: A total of 561 patients with genotype 1/6 CHC were enrolled, among whom 529 received treatment; 90.9% of these patients had genotype 1 CHC. The data of the full analysis set showed that SVR rate was 69.80% (95% CI 65.00%-74.60%) in the trial group and 74.16% (95% CI 67.73%-80.59%) in the control group (P = 0.297 0). The data of the per protocol set (PPS) showed that SVR rate was 80.63% (95% CI 76.04%-85.23%) in the trial group and 81.33% (95% CI 75.10%-87.57%) in the control group (P = 0.849 8), and the 95% CI of rate difference conformed to the non-inferiority standard. The analysis of the PPS population showed that of all subjects, 47.9% achieved rapid virologic response, with a positive predictive value of 93.8%. The incidence rate of adverse events was 96.30% in the trial group and 94.94% in the control group, and the incidence rate of serious adverse events was 5.13% in the trail group and 5.06% in the control group. Conclusion In the regimen of Peg-IFN-α combined with ribavirin for the treatment of genotype 1/6 CHC, the new investigational drug Peg-IFN-α-2b(Y shape, 40 kD) has comparable clinical effect and safety to the control drug Peg-IFN-α-2a.