The core pathogenesis of pediatric IgA vasculitis （IgAV） is considered to involve qi movement disorder， with the failure of clear-turbid separation and internal generation of turbid pathogens. Accordingly， the viewpoint of "qi disorder causing turbidity" is proposed. In clinical practice， the disease is divided into the acute， persistent， and stable stages. The acute stage is characterized by impaired dispersion of wei （卫） qi and the internal generation of heat turbidity， while the persistent stage by congestion of ying （营） qi and retention of stasis-turbidity， and the stable stage by depletion of pectoral qi with lingering latent turbidity. In clinical practice， regulating qi and transforming turbidity is taken as the basic therapeutic principle， and the self-formulated Tiaoqi Huazhuo Decoction （调气化浊汤） is used as the basic prescription for staged treatment with flexible modifications. Specifically， the acute stage is treated with the method of dispersing wei qi and clearing heat-turbidity； the persistent stage is recommended to harmonize ying qi and purging stasis-turbidity； and the stable stage should focus on securing pectoral qi and venting latent turbidity.

[This corrects the article DOI: 10.1016/j.apsb.2022.05.019.].

Objective:To evaluate the efficacy of eculizumab in treating hematopoietic stem cell transplantation-associated thrombotic microangiopathy (TA-TMA) .Methods:This retrospective study included 11 patients who developed TA-TMA after allogeneic hematopoietic stem cell transplantation and subsequently received eculizumab treatment at Peking University People′s Hospital between June 2018 and May 2024. The incidence of TA-TMA, treatment details, and clinical outcomes were analyzed.Results:Among the 11 included patients [4 males, 7 females; median age: 29 years (range: 9-56) ], underlying diseases were severe aplastic anemia (SAA) in 5 patients, acute lymphoblastic leukemia (ALL) in 3 patients, and acute myeloid leukemia (AML) in 3 patients. The median time to TA-TMA diagnosis was 48 days post-transplantation (range: 4-213 days), and all patients met the diagnostic criteria for high-risk TA-TMA. The median interval from TA-TMA diagnosis to the initiation of eculizumab treatment was 12 days (range: 1-56 days). Patients received a median of 3 doses of eculizumab (range: 1-14). Ten of the 11 patients were assessed as having no response (NR) to eculizumab at the end of treatment or at death. One patient achieved a partial response (PR) but subsequently died after TA-TMA relapsed due to infection. At the last follow-up, all patients were either lost to follow-up or had died. The median follow-up duration was 88 days (range: 33-326 days), and the median time from TA-TMA diagnosis to the last follow-up was 31 days (range: 21-113 days) .Conclusion:Eculizumab demonstrated poor efficacy in this TA-TMA cohort. This might be attributable to the critical and complex condition of the patients, delayed initiation of eculizumab treatment, and insufficient dosage.

Objective:To evaluate the diagnostic value of targeted next-generation sequencing (tNGS) of throat swab samples for detecting community-acquired respiratory viruses (CARV) in patients with hematological diseases.Methods:Clinical and laboratory data from 64 episodes involving patients with hematological diseases and suspected infections—who underwent both pharyngeal swab tNGS and CARV polymerase chain reaction (PCR) testing concurrently—were retrospectively analyzed. The cases were drawn from the Department of Hematology, Peking University People’s Hospital, between September 2023 and April 2024. Concordance between tNGS and CARV PCR results, as well as the diagnostic performance of tNGS in detecting CARV, were evaluated.Results:Among the 64 episodes, 29 were clinically diagnosed with respiratory tract infections, including one case of cytomegalovirus pneumonia and 28 CARV-positive cases. The remaining 35 episodes involved patients with fever or respiratory symptoms attributed to other causes, including 14 with extrapulmonary infections and 21 with noninfectious etiologies. The median follow-up duration was 215.5 days (range: 7-271 days). PCR detected 24 strains of seven CARV types, whereas tNGS detected 25 strains of eight CARV types. Using PCR results as the reference standard, the sensitivity, specificity, positive predictive value, negative predictive value and accuracy of tNGS were 85.0%, 88.6%, 77.3%, 92.9%, and 87.5%, respectively. The two methods showed good concordance (Kappa=0.717, P<0.001) . Conclusion:Pharyngeal swab tNGS may serve as a viable alternative to PCR for diagnosing CARV infections in patients with hematological diseases.

Objective:To evaluate the efficacy of eculizumab in treating hematopoietic stem cell transplantation-associated thrombotic microangiopathy (TA-TMA) .Methods:This retrospective study included 11 patients who developed TA-TMA after allogeneic hematopoietic stem cell transplantation and subsequently received eculizumab treatment at Peking University People′s Hospital between June 2018 and May 2024. The incidence of TA-TMA, treatment details, and clinical outcomes were analyzed.Results:Among the 11 included patients [4 males, 7 females; median age: 29 years (range: 9-56) ], underlying diseases were severe aplastic anemia (SAA) in 5 patients, acute lymphoblastic leukemia (ALL) in 3 patients, and acute myeloid leukemia (AML) in 3 patients. The median time to TA-TMA diagnosis was 48 days post-transplantation (range: 4-213 days), and all patients met the diagnostic criteria for high-risk TA-TMA. The median interval from TA-TMA diagnosis to the initiation of eculizumab treatment was 12 days (range: 1-56 days). Patients received a median of 3 doses of eculizumab (range: 1-14). Ten of the 11 patients were assessed as having no response (NR) to eculizumab at the end of treatment or at death. One patient achieved a partial response (PR) but subsequently died after TA-TMA relapsed due to infection. At the last follow-up, all patients were either lost to follow-up or had died. The median follow-up duration was 88 days (range: 33-326 days), and the median time from TA-TMA diagnosis to the last follow-up was 31 days (range: 21-113 days) .Conclusion:Eculizumab demonstrated poor efficacy in this TA-TMA cohort. This might be attributable to the critical and complex condition of the patients, delayed initiation of eculizumab treatment, and insufficient dosage.

Objective:To evaluate the diagnostic value of targeted next-generation sequencing (tNGS) of throat swab samples for detecting community-acquired respiratory viruses (CARV) in patients with hematological diseases.Methods:Clinical and laboratory data from 64 episodes involving patients with hematological diseases and suspected infections—who underwent both pharyngeal swab tNGS and CARV polymerase chain reaction (PCR) testing concurrently—were retrospectively analyzed. The cases were drawn from the Department of Hematology, Peking University People’s Hospital, between September 2023 and April 2024. Concordance between tNGS and CARV PCR results, as well as the diagnostic performance of tNGS in detecting CARV, were evaluated.Results:Among the 64 episodes, 29 were clinically diagnosed with respiratory tract infections, including one case of cytomegalovirus pneumonia and 28 CARV-positive cases. The remaining 35 episodes involved patients with fever or respiratory symptoms attributed to other causes, including 14 with extrapulmonary infections and 21 with noninfectious etiologies. The median follow-up duration was 215.5 days (range: 7-271 days). PCR detected 24 strains of seven CARV types, whereas tNGS detected 25 strains of eight CARV types. Using PCR results as the reference standard, the sensitivity, specificity, positive predictive value, negative predictive value and accuracy of tNGS were 85.0%, 88.6%, 77.3%, 92.9%, and 87.5%, respectively. The two methods showed good concordance (Kappa=0.717, P<0.001) . Conclusion:Pharyngeal swab tNGS may serve as a viable alternative to PCR for diagnosing CARV infections in patients with hematological diseases.

Objective:To conjecture the correlation between fetal hydrothorax(FHT)and pregnancy outcome through the analysis of 95 fetuses with hydrothorax and their mothers.Methods:In this case series study, 95 FHT patients admitted to the Third Affiliated Hospital of Zhengzhou University from January 2016 to October 2022 were retrospectively analyzed.According to the pregnancy outcome, these patients were divided into the induced labor group (13 patients) and the delivery group (82 patients). General data and genetic examinations of patients in the induced labor group were analyzed to explore their pathogenesis and genetics.According to the neonatal Apgar score, patients in the delivery group were divided into the normal group and the asphyxia group.Fifteen indicators including the maternal age, gestational week at first diagnosis, maternal complications, FHT location, FHT severity, FHT outcome during pregnancy, fetal ascites, hydrops fetalis, other abnormal fetal structures, amniotic fluid volume, intrauterine treatment, gestational week of delivery, mode of delivery, sex of the newborn, and newborn birth weight in the 2 groups were comparatively analyzed by the chi-square test.With the neonatal Apgar score as the dependent variable, variables that had statistical significance during the univariate analysis were included in the regression analysis, and a multivariate binary Logistic regression equation was established to explore the factors affecting the pregnancy outcome.Results:There were significant differences in the FHT outcome during pregnancy (16/22/13 cases vs.2/18/11 cases, χ2=6.994, P=0.030), FHT severity (27/24 cases vs.9/22 cases, χ2=4.475, P=0.034), hydrops fetalis (14/37 cases vs.23/8 cases, χ2=17.012, P=0.001), amniotic fluid volume (21/30 cases vs.24/7 cases, χ2=10.228, P=0.001), intrauterine treatment (19/32 cases vs.2/29 cases, χ2=9.603, P=0.002), gestational week of delivery[(38.15±2.05) weeks vs.(34.83±3.14) weeks, t=5.748, P=0.001], and newborn birth weight[(3 325.00±637.41) g vs.(2 714.58±837.99) g, t=3.727, P=0.001]between the normal and asphyxia groups(all P<0.05). Among them, hydrops fetalis ( OR=7.070, P=0.020) and severe FHT ( OR=6.927, P=0.043) were risk factors for neonatal Apgar scores.Intrauterine treatment ( OR=0.062, P=0.027) and gestational week of delivery( OR=0.577, P=0.022) could be used as a protective factor for neonatal Apgar scores. Conclusions:Diagnosis of FHT during the early gestational weeks and multiple fetal structural abnormalities, especially hydrops fetalis, have higher probabilities of abnormal genetic examinations, and the fetal prognosis was poor.It is recommended to carry out chromosomal karyo type and microarray tests, as well as whole exome sequencing and detection of genetic syndromes(if necessary), to avoid unnecessary fetal treatment and improve the survival rate of perinatal infants after intrauterine treatment.The poor prognosis is related to hydrops fetalis and severe FHT; however, timely intrauterine treatment and prolonging pregnancy can improve the pregnancy outcome and increase the survival rate of perinatal infants.

Objective:To further improve the understanding of paroxysmal nocturnal hemoglobinuria (PNH), we retrospectively analyzed and summarized the clinical characteristics, treatment status, and survival status of patients with PNH in Zhejiang Province.Methods:This study included 289 patients with PNH who visited 20 hospitals in Zhejiang Province. Their clinical characteristics, comorbidity, laboratory test results, and medications were analyzed and summarized.Results:Among the 289 patients with PNH, 148 males and 141 females, with a median onset age of 45 (16-87) years and a peak onset age of 20-49 years (57.8% ). The median lactic dehydrogenase (LDH) level was 1 142 (604-1 925) U/L. Classified by type, 70.9% (166/234) were classical, 24.4% (57/234) were PNH/bone marrow failure (BMF), and 4.7% (11/234) were subclinical. The main clinical manifestations included fatigue or weakness (80.8%, 235/289), dizziness (73.4%, 212/289), darkened urine color (66.2%, 179/272), and jaundice (46.2%, 126/270). Common comorbidities were hemoglobinuria (58.7% ), renal dysfunction (17.6% ), and thrombosis (15.0% ). Moreover, 82.3% of the patients received glucocorticoid therapy, 70.9% required blood transfusion, 30.7% used immunosuppressive agents, 13.8% received anticoagulant therapy, and 6.3% received allogeneic hematopoietic stem cell transplantation. The 10-year overall survival (OS) rate was 84.4% (95% CI 78.0% -91.3% ) . Conclusion:Patients with PNH are more common in young and middle-aged people, with a similar incidence rate between men and women. Common clinical manifestations include fatigue, hemoglobinuria, jaundice, renal dysfunction, and recurrent thrombosis. The 10-year OS of this group is similar to reports from other centers in China.

Objective:To evaluate the efficacy and safety of hypomethylating agents (HMA) in patients with myelodysplastic syndromes (MDS) .Methods:A total of 409 MDS patients from 45 hospitals in Zhejiang province who received at least four consecutive cycles of HMA monotherapy as initial therapy were enrolled to evaluate the efficacy and safety of HMA. Mann-Whitney U or Chi-square tests were used to compare the differences in the clinical data. Logistic regression and Cox regression were used to analyze the factors affecting efficacy and survival. Kaplan-Meier was used for survival analysis. Results:Patients received HMA treatment for a median of 6 cycles (range, 4-25 cycles) . The complete remission (CR) rate was 33.98% and the overall response rate (ORR) was 77.02%. Multivariate analysis revealed that complex karyotype ( P=0.02, OR=0.39, 95% CI 0.18-0.84) was an independent favorable factor for CR rate. TP53 mutation ( P=0.02, OR=0.22, 95% CI 0.06-0.77) was a predictive factor for a higher ORR. The median OS for the HMA-treated patients was 25.67 (95% CI 21.14-30.19) months. HMA response ( P=0.036, HR=0.47, 95% CI 0.23-0.95) was an independent favorable prognostic factor, whereas complex karyotype ( P=0.024, HR=2.14, 95% CI 1.10-4.15) , leukemia transformation ( P<0.001, HR=2.839, 95% CI 1.64-4.92) , and TP53 mutation ( P=0.012, HR=2.19, 95% CI 1.19-4.07) were independent adverse prognostic factors. There was no significant difference in efficacy and survival between the reduced and standard doses of HMA. The CR rate and ORR of MDS patients treated with decitabine and azacitidine were not significantly different. The median OS of patients treated with decitabine was longer compared with that of patients treated with azacitidine (29.53 months vs 20.17 months, P=0.007) . The incidence of bone marrow suppression and pneumonia in the decitabine group was higher compared with that in the azacitidine group. Conclusion:Continuous and regular use of appropriate doses of hypomethylating agents may benefit MDS patients to the greatest extent if it is tolerated.

Objective To explore the trends and patterns of changes in airway inflammation,mitochondrial function,and arginine levels in patients with different degrees of asthma.Methods A total of 122 patients with acute asthma admitted to the Affiliated Traditional Chinese Medicine Hospital of Xinjiang Medical University from November 2016 to May 2017 were selected as the research subjects.According to the severity grading criteria,the patients were divided into the intermittent state group(n=45),mild asthma group(n=23),moderate asthma group(n=30),and severe asthma group(n=24).Another 44 healthy individuals who underwent physical examinations at the Medical Examination Center of the Affiliated Traditional Chinese Medicine Hospital of Xinjiang Medical University from November 2016 to May 2017 were selected as the healthy control group.About 18-20 mL of venous blood was extracted from each group of subjects.The levels of L-arginine(L-Arg)and asymmetric dimethylarginine(ADMA)were detected by high-performance liquid chromatography-tandem mass spectrometry,and the levels of type 1 protein arginine methyltransferase(PRMT1),dimethylarginine dimethylaminohydrolase 1(DDAH1),reactive oxygen species(ROS),thiobarbituric acid reactive substances(TBARS),8-isoprostaglandin(8-iso),lipid peroxide(LPO),and malondialdehyde(MDA)were detected by enzyme-linked immunosorbent assay.Platelets and mitochondria were extracted from each group of subjects.The platelet mitochondrial membrane potential(MMP)was measured using JC-1 flow cytometry,and the mitochondrial adenosine triphosphate(ATP)and cytochrome C oxidase(COX)activity were detected.Results The level of 8-iso in plasma of patients in the intermittent state group,mild asthma group,moderate asthma group,and severe asthma group were significantly higher than that in the healthy control group(P＜0.05);the level of 8-iso in plasma of patients in the severe asthma group was significantly higher than that in the intermittent state group,mild asthma group,and moderate asthma group(P＜0.05);the level of 8-iso in plasma of patients in the intermittent state group,mild asthma group,and moderate asthma group showed an increasing trend,but there was no statistically significant difference(P＞0.05).There was no statistically significant difference in the TBARS level in the plasma of subjects in the five groups(P＞0.05).The levels of ROS and MDA in plasma of patients in the intermittent state group,mild asthma group,moderate asthma group,and severe asthma group were significantly higher than those in the healthy control group(P＜0.05);the levels of ROS and MDA in plasma of patients in the moderate asthma group and severe asthma group were significantly higher than those in the intermittent state group and mild asthma group(P＜0.05);there was no statistically significant difference in plasma ROS and MDA levels between the intermittent state group and the mild asthma group(P＞0.05),and there was no statistically significant difference in plasma ROS and MDA levels between the moderate asthma group and the severe asthma group(P＞0.05).The plasma LPO level of patients in the intermittent state group,mild asthma group,moderate asthma group,and severe asthma group was significantly higher than that in the healthy control group(P＜0.05);the plasma LPO level of patients in the moderate asthma group and severe asthma group was significantly higher than that in the intermittent state group(P＜0.05);there was no statistically significant difference in plasma LPO level of patients in the mild asthma group,moderate asthma group,and severe asthma group(P＞0.05).The mitochondrial MMP,ATP,and COX activity in the intermittent state group,mild asthma group,moderate asthma group,and severe asthma group were significantly lower than those in the healthy control group(P＜0.05);there was no statistically significant difference in mitochondrial MMP,ATP,and COX activity of patients in the intermittent state group,mild asthma group,moderate asthma group,and severe asthma group(P＞0.05).The levels of plasma L-Arg,the ratio of L-Arg/ADMA,and DDAH1 in the intermittent state group,mild asthma group,moderate asthma group,and severe asthma group were significantly lower than those in the healthy control group(P＜0.05);there was no statistically significant difference in plasma L-Arg,the ratio of L-Arg/ADMA,and DDAH1 levels of patients in the intermittent state group,mild asthma group,moderate asthma group,and severe asthma group(P＞0.05).The plasma ADMA and PRMT1 levels showed no statistically significant difference among the five groups of patients(P＞0.05).Conclusion Asthma patients exhibit mitochondrial dysfunction and abnormal arginine metabolism at any stage of the disease.Although there is no statistically significant difference in the change trend at the cellular micro level among patients with different stages of asthma,the trend of changes in oxidative damage in the body is consistent with the aggravation of the disease.