1.Analysis of Animal Models of Dry Age-related Macular Degeneration Based on Clinical Disease-syndrome Characteristics of Traditional Chinese and Western Medicine
Xiaoyu LI ; Lina LIANG ; Yun GAO ; Jiahao LI ; Jianying YANG ; Xiaoshan ZHANG ; Honghao BI ; Menglu MIAO ; Huiyi GUO
Chinese Journal of Experimental Traditional Medical Formulae 2026;32(3):191-197
ObjectiveAge-related macular degeneration (AMD) is one of the leading causes of low vision and blindness in people over 50 years old, and dry AMD (dAMD) is one type for which there is currently no clear treatment. On the basis of the diagnosis and clinical characteristics of dAMD in traditional Chinese and Western medicine, this paper evaluated the fitting degrees of existing animal models of dAMD with clinical characteristics according to the evaluation methods of animal models, and put forward suggestions and prospects. MethodsLiterature on animal models of dAMD was searched against database, and the characteristics of the models were assigned according to the diagnosis criteria of diseases and syndromes of traditional Chinese and Western medicine, and the fitting degrees of the models with clinical characteristics were analyzed and evaluated. ResultsAt present, the animal models of dAMD are mainly established targeting complement factors, chemokines, oxidative damage, lipid/glucose metabolism, and natural strains. Most of the models can simulate the major pathological changes of dAMD, showing the fitting degree of 25%-50% with clinical characteristics in Western medicine. However, the evaluation of traditional Chinese medicine (TCM) syndromes, especially the evaluation of secondary syndromes, is missing, and the models present low fitting degrees with the clinical characteristics in TCM. ConclusionExisting animal models of dAMD are mostly established under the guidance of Western diagnostic standards, which reproduce the main disease characteristics of Western medicine and lack observation of TCM syndromes. Future studies can pay attention to the intervention factors and evaluation systems of spleen deficiency Qi deficiency and liver-kidney Yin deficiency syndrome and build the animal model of dAMD with integration of disease and syndrome based on clinical characteristics of traditional Chinese and Western medicine.
2.Analysis of Animal Models of Dry Age-related Macular Degeneration Based on Clinical Disease-syndrome Characteristics of Traditional Chinese and Western Medicine
Xiaoyu LI ; Lina LIANG ; Yun GAO ; Jiahao LI ; Jianying YANG ; Xiaoshan ZHANG ; Honghao BI ; Menglu MIAO ; Huiyi GUO
Chinese Journal of Experimental Traditional Medical Formulae 2026;32(3):191-197
ObjectiveAge-related macular degeneration (AMD) is one of the leading causes of low vision and blindness in people over 50 years old, and dry AMD (dAMD) is one type for which there is currently no clear treatment. On the basis of the diagnosis and clinical characteristics of dAMD in traditional Chinese and Western medicine, this paper evaluated the fitting degrees of existing animal models of dAMD with clinical characteristics according to the evaluation methods of animal models, and put forward suggestions and prospects. MethodsLiterature on animal models of dAMD was searched against database, and the characteristics of the models were assigned according to the diagnosis criteria of diseases and syndromes of traditional Chinese and Western medicine, and the fitting degrees of the models with clinical characteristics were analyzed and evaluated. ResultsAt present, the animal models of dAMD are mainly established targeting complement factors, chemokines, oxidative damage, lipid/glucose metabolism, and natural strains. Most of the models can simulate the major pathological changes of dAMD, showing the fitting degree of 25%-50% with clinical characteristics in Western medicine. However, the evaluation of traditional Chinese medicine (TCM) syndromes, especially the evaluation of secondary syndromes, is missing, and the models present low fitting degrees with the clinical characteristics in TCM. ConclusionExisting animal models of dAMD are mostly established under the guidance of Western diagnostic standards, which reproduce the main disease characteristics of Western medicine and lack observation of TCM syndromes. Future studies can pay attention to the intervention factors and evaluation systems of spleen deficiency Qi deficiency and liver-kidney Yin deficiency syndrome and build the animal model of dAMD with integration of disease and syndrome based on clinical characteristics of traditional Chinese and Western medicine.
3.Clinical efficacy of internal decompression based on white matter tract preservation in treatment of malignant middle cerebral artery infarction
Yanli ZHANG ; Menglu LI ; Jiankai ZHAO ; Jingmin DOU ; Fei ZHANG ; Baoming JIA ; Guoqiang FENG ; Hongbin KU
Chinese Journal of Nervous and Mental Diseases 2025;51(10):596-600
Objective To investigate the clinical efficacy of internal decompression based on white matter tract preservation in the treatment of malignant middle cerebral artery infarction(MMCAI).Methods A retrospective analysis was conducted on 54 patients with MMCAI.Patients were divided into a study group(n=26)and a control group(n=28)according to the surgical approach.Patients in the study group underwent preoperative fusion of CT,CTP,DWI,and DTI imaging data within a neuronavigation system.This fusion visualized the spatial relationships between the infarct core(IC),ischemic penumbra,and the corticospinal tract(CST).Subsequently,IC resection combined with decompressive craniectomy(DC)was performed while protecting the CST.Patients in the control group underwent DC alone.Key outcome measures included:changes in fractional anisotropy(FA)within the affected CST projection area at 1 month postoperatively;and 6-month postoperative mRS score,mortality,and surgical complications at 6 months postoperatively.Results At 1 month postoperatively,FA in the affected CST projection area were significantly higher in the study group than in the control group(0.092±0.013 vs.0.082±0.008,P<0.05).At the 6-month follow-up,the postoperative mRS score in the study group was significantly lower than that in the control group[2.3(1.3,4.5)vs.3.9(2.4,5.5),P<0.05]and a lower mortality rate(11.5%vs.39.3%,P<0.05)compared to the control group.However,there were no statistically significant differences between the two groups in the incidence of postoperative intracranial hemorrhage,intracranial infection,or epilepsy(P>0.05).Conclusion Internal decompression based on white matter tract protection combined with DC can reduce mortality and contribute to improving function outcomes in patients with MMCAI.
4.Correlation between complement deposition levels on blood cells and clinical biomarkers in patients with paroxysmal nocturnal hemoglobinuria
Menglu ZHANG ; Xuan WANG ; Chen YANG ; Miao CHEN ; Bing HAN
Chinese Journal of Hematology 2025;46(4):314-320
Objective:To explore the complement deposition levels on blood cell surfaces in patients with paroxysmal nocturnal hemoglobinuria (PNH) and evaluate their association with clinical manifestations.Methods:This study enrolled patients with PNH, who had not been treated with complement inhibitors and appeared at Peking Union Medical College Hospital from February 2021 to February 2023. The clinical information of participants was retrospectively recorded, and peripheral blood samples were collected. Gender- and age-matched normal controls (NC) were recruited accordingly. C5b-9, C3, C4b, and factor B (FB) deposition levels on peripheral red blood cells, white blood cells, and platelets were detected with flow cytometry. The correlation between complement deposition levels and clinical symptoms was analyzed.Results:This study involved 73 patients with PNH, including 42 (57.5%) males, with a median age of 36 (range: 14-76) years. 16 matched NC were collected. Among patients with PNH, 36 (49.3%) had classical PNH and 37 (50.7%) had aplastic anemia-PNH syndrome. Thromboembolic events (TEE) occurred in 18 (24.7%) patients. The median HGB, absolute reticulocyte count (Ret), and lactate dehydrogenase of PNH patients were 76 (37-116) g/L, 181.0 (45.9-495.8) ×10 9/L, and 1 875 (377 - 5 509) U/L, respectively. The median number of Flaer-negative white blood cells was 94.0% (13.0% - 99.9%) ; the median CD59 negative red blood cells was 46.7% (9.0% - 93.0%). The deposition of C5b-9, C3, C4b, and FB on red blood cells, white blood cells, and platelets in patients with PNH was significantly higher than that in NC (all P<0.05). C5b-9 deposition level was significantly higher than that of C3, C4b, and FB on all three blood cell lineages in PNH patients (all P<0.01). The deposition of all complement fragments on red blood cells was significantly lower than that on white blood cells and platelets (all P<0.01). C5b-9 deposition on red blood cells was positively correlated with Ret in PNH patients ( P=0.005). C3 ( P=0.001) and C4b ( P=0.017) deposition levels on white blood cells and C3 deposition on platelets ( P=0.002) in patients with TEE history were lower than those without. Conclusions:C5b-9, C3, C4b, and FB deposition levels on all three blood cells in patients with PNH were higher than NC. Increased C5b-9 on red blood cells may indicate active hemolysis. Reduced C3 and C4b levels on white blood cells and low C3 deposition on platelets may indicate TEE risk.
5.Efficacy analysis of infarct core resection combined with decompressive craniectomy based on corticospinal tract protection for massive cerebral infarction with malignant brain edema
Yanli ZHANG ; Jingmin DOU ; Menglu LI ; Fei ZHANG ; Baoming JIA ; Jiankai ZHAO ; Hongbin KU ; Guoqiang FENG
Chinese Journal of Cerebrovascular Diseases 2025;22(6):383-394
Objective To investigate the effectiveness and safety of infarct core resection combined with decompressive craniectomy(DC)based on corticospinal tract(CST)protection in the treatment of massive cerebral infarction(MCI)with malignant brain edema.Methods This study retrospectively enrolled MCI patients with malignant brain edema who underwent internal decompression combined with DC at Xingtai Central Hospital from January 2021 to June 2024.The enrolled patients were divided into a control group and an experimental group base on the intracranial internal decompression method used.All patients underwent CT perfusion(CTP),CT angiography(CTA),diffusion-weighted imaging(DWI),and diffusion tensor imaging(DTI)within 24 h of admission.Preoperative imaging data was automatically processed using an artificial intelligence diagnostic system.For the experimental group,the imaging data was fused within a neuro-navigation system preoperatively to visualize the spatial relationships between the infarct core,ischemic penumbra,and CST and infarct core resection combined with DC was performed while protecting the CST through neuro-navigation.The control group underwent anterior temporal lobectomy combined with DC.Baseline and clinical data were collected from both groups,including gender,age,smoking history,alcohol consumption history,diabetes,hypertension,hyperlipidemia,hyperhomocysteinemia,atrial fibrillation history,responsible occluded vessel(internal carotid artery,middle cerebral artery),preoperative infarct volume on DWI,preoperative ischemic penumbra volume,preoperative the National Institutes of Health stroke scale(NIHSS)score,time from onset to surgery,intraoperative procedure duration,intraoperative blood loss,preoperative and 1-month postoperative fraction anisotropy(FA)values of the CST on the affected side,modified Rankin scale(mRS)score at 6 months postoperatively,and surgery-related complications within 1 month postoperatively(intracranial hemorrhage[operative site oozing,hemorrhagic transformation]and intracranial infection[surgical incision site infection,empyema,brain abscess,meningitis]).6-month follow-up after surgery were conducted through outpatient visit or telephone calls and prognosis of patients was evaluated using the mRS(with mRS of 0-3 defined as good prognosis,4-6 as poor prognosis,and 6 indicating death).The effectiveness indicators included FA value of the affected CST at 1 month postoperatively,good prognosis rate after surgery at 6 months,and 6-month mortality rate after surgery.The safety indicators included the incidence rates of surgical complications(intracranial hemorrhage and infection)within 1 month postoperatively.Based on preoperative DTI images,all patients were further divided into a CST-intact(infarct core did not invade CST,CST morphology intact or deformed/shifted)and a CST-damaged(infarct core invaded CST,CST disrupted or interrupted)subgroup for analysis.Results A total of 62patients(37 males,25 females,age 49-60 years,mean[55±4]years)were enrolled in this study.With 28 patients in the experimental group and 34 in the control group.(1)No significant differences were found in baseline or clinical data between the experimental and control groups(all P>0.05),and the reoperative FA values of the affected CST were showed no significant differences(P=0.588).(2)The efficacy and safety metrics were evaluated.For the efficacy indices,at 1 month after the surgery,FA values of the affected CST increased significantly compared to preoperative values in both groups(0.409±0.051 vs.0.312±0.052 in the experimental group,and,0.381±0.048 vs.0.319±0.049 in control group;both P<0.05),and the FA value was significantly higher in the experimental group than that in the control group(0.409±0.051 vs.0.381±0.048,P=0.030).At the 6-month follow-ups,the good prognosis rate was significantly higher in the experimental group than that in the control group(39.3%[11/28]vs.14.7%[5/34],P=0.028).No significant difference in the 6-month mortality rate were observed between the two groups(P=0.787).For the safety indices,no significant differences were found in the incidence rates of intracranial hemorrhage or intracranial infection within 1 month postoperatively between the two groups(both P>0.05).(3)For further subgroup analysis,no significant differences were found in baseline or clinical data between the CST-damaged subgroup and the CST-intact subgroup in both the experimental and control groups(all P>0.05).In CST-intact subgroup,FA values of the affected CST increased significantly at 1 month postoperatively compared to preoperatively in the study group(0.428±0.047 vs.0.342±0.045,P<0.05)and the control group(0.401±0.051 vs.0.347±0.048,P<0.05).While in the CST-damaged subgroup,no significant differences were found in FA value of the affected CST 1 month postoperatively compared with that preoperatively in both the experimental and control groups(bothP>0.05).A significantly higher FA values 1 month postoperatively(0.428±0.047 vs.0.401±0.051,P=0.036)and good prognosis rate(9/12 vs.4/16,P=0.020)were observed in the CST-intact subgroup of the experimental group comparing with that of the control group,while there was no statistically significant difference in the 6-month mortality rate between the groups within the CST-intact subgroup(P=1.000).There were no statistically significant differences between the experimental group and the control group in both efficacy and safety indices within the CST-damaged subgroup(all P>0.05).Conclusions Infarct core resection combining DC with CST protection demonstrates superior neurological functional improvement in comparison with anterior temporal lobectomy combining DC in treating MCI with malignant brain edema,particularly for patients with an intact CST before surgery(as indicated in patients'preoperative imaging results).This(infarct core resection combining DC with CST protection)approach does not increase the incidence of surgical complications.Prospective large sample controlled studies are required for further validation.
6.Correlation Between Fractional Area Change of Left Ventricle Measured by Cardiac Magnetic Resonance and Myocardial Circumferential Strain
Menglu LI ; Heng ZHANG ; Chunjie WANG ; Fuzhuang JIANG ; Kaizhou YANG ; Yuanyuan XING ; Fangmin PEI ; Liuquan CHENG
Chinese Journal of Medical Imaging 2025;33(7):739-744
Purpose To compare the similarity between the short-axis left ventricular cavity fractional area change measured by cardiac magnetic resonance and myocardial circumferential strain.Materials and Methods Forty cases of cardiomyopathy with different phenotypes and high-quality cardiac magnetic resonance images in PACS data center of the Sixth Medical Center of Chinese PLA General Hospital were retrospectively selected.All patients underwent cine imaging on a 3.0T MRI scanner.The endocardial and epicardial areas of each short-axis left ventricular slice were measured using Medviso Segment software to calculate slice-level fractional area change and global fractional area change.Slice circumferential strain and global circumferential strain were measured using Circle CVI42 software.Linear regression analysis was performed to assess correlations between global circumferential strain and global fractional area change,as well as slice circumferential strain and slice-level fractional area change.Results Both global circumferential strain and global fractional area change,as well as slice circumferential strain and slice-level fractional area change,exhibited positive correlations(all r>0.95).Linear regression demonstrated significant relationships(regression coefficients:2.40-3.16,P<0.05),with coefficient magnitudes related to left ventricular cavity radius.After normalization,circumferential strain and fractional area change curves showed identical standard deviations.Conclusion Short-axis left ventricular cavity fractional area change and myocardial circumferential strain display significant positive correlations at both slice and global levels,with similar curve morphology.These findings suggest that circumferential strain and fractional area change possess comparable statistical value in assessing cardiac function.
7.Efficacy analysis of infarct core resection combined with decompressive craniectomy based on corticospinal tract protection for massive cerebral infarction with malignant brain edema
Yanli ZHANG ; Jingmin DOU ; Menglu LI ; Fei ZHANG ; Baoming JIA ; Jiankai ZHAO ; Hongbin KU ; Guoqiang FENG
Chinese Journal of Cerebrovascular Diseases 2025;22(6):383-394
Objective To investigate the effectiveness and safety of infarct core resection combined with decompressive craniectomy(DC)based on corticospinal tract(CST)protection in the treatment of massive cerebral infarction(MCI)with malignant brain edema.Methods This study retrospectively enrolled MCI patients with malignant brain edema who underwent internal decompression combined with DC at Xingtai Central Hospital from January 2021 to June 2024.The enrolled patients were divided into a control group and an experimental group base on the intracranial internal decompression method used.All patients underwent CT perfusion(CTP),CT angiography(CTA),diffusion-weighted imaging(DWI),and diffusion tensor imaging(DTI)within 24 h of admission.Preoperative imaging data was automatically processed using an artificial intelligence diagnostic system.For the experimental group,the imaging data was fused within a neuro-navigation system preoperatively to visualize the spatial relationships between the infarct core,ischemic penumbra,and CST and infarct core resection combined with DC was performed while protecting the CST through neuro-navigation.The control group underwent anterior temporal lobectomy combined with DC.Baseline and clinical data were collected from both groups,including gender,age,smoking history,alcohol consumption history,diabetes,hypertension,hyperlipidemia,hyperhomocysteinemia,atrial fibrillation history,responsible occluded vessel(internal carotid artery,middle cerebral artery),preoperative infarct volume on DWI,preoperative ischemic penumbra volume,preoperative the National Institutes of Health stroke scale(NIHSS)score,time from onset to surgery,intraoperative procedure duration,intraoperative blood loss,preoperative and 1-month postoperative fraction anisotropy(FA)values of the CST on the affected side,modified Rankin scale(mRS)score at 6 months postoperatively,and surgery-related complications within 1 month postoperatively(intracranial hemorrhage[operative site oozing,hemorrhagic transformation]and intracranial infection[surgical incision site infection,empyema,brain abscess,meningitis]).6-month follow-up after surgery were conducted through outpatient visit or telephone calls and prognosis of patients was evaluated using the mRS(with mRS of 0-3 defined as good prognosis,4-6 as poor prognosis,and 6 indicating death).The effectiveness indicators included FA value of the affected CST at 1 month postoperatively,good prognosis rate after surgery at 6 months,and 6-month mortality rate after surgery.The safety indicators included the incidence rates of surgical complications(intracranial hemorrhage and infection)within 1 month postoperatively.Based on preoperative DTI images,all patients were further divided into a CST-intact(infarct core did not invade CST,CST morphology intact or deformed/shifted)and a CST-damaged(infarct core invaded CST,CST disrupted or interrupted)subgroup for analysis.Results A total of 62patients(37 males,25 females,age 49-60 years,mean[55±4]years)were enrolled in this study.With 28 patients in the experimental group and 34 in the control group.(1)No significant differences were found in baseline or clinical data between the experimental and control groups(all P>0.05),and the reoperative FA values of the affected CST were showed no significant differences(P=0.588).(2)The efficacy and safety metrics were evaluated.For the efficacy indices,at 1 month after the surgery,FA values of the affected CST increased significantly compared to preoperative values in both groups(0.409±0.051 vs.0.312±0.052 in the experimental group,and,0.381±0.048 vs.0.319±0.049 in control group;both P<0.05),and the FA value was significantly higher in the experimental group than that in the control group(0.409±0.051 vs.0.381±0.048,P=0.030).At the 6-month follow-ups,the good prognosis rate was significantly higher in the experimental group than that in the control group(39.3%[11/28]vs.14.7%[5/34],P=0.028).No significant difference in the 6-month mortality rate were observed between the two groups(P=0.787).For the safety indices,no significant differences were found in the incidence rates of intracranial hemorrhage or intracranial infection within 1 month postoperatively between the two groups(both P>0.05).(3)For further subgroup analysis,no significant differences were found in baseline or clinical data between the CST-damaged subgroup and the CST-intact subgroup in both the experimental and control groups(all P>0.05).In CST-intact subgroup,FA values of the affected CST increased significantly at 1 month postoperatively compared to preoperatively in the study group(0.428±0.047 vs.0.342±0.045,P<0.05)and the control group(0.401±0.051 vs.0.347±0.048,P<0.05).While in the CST-damaged subgroup,no significant differences were found in FA value of the affected CST 1 month postoperatively compared with that preoperatively in both the experimental and control groups(bothP>0.05).A significantly higher FA values 1 month postoperatively(0.428±0.047 vs.0.401±0.051,P=0.036)and good prognosis rate(9/12 vs.4/16,P=0.020)were observed in the CST-intact subgroup of the experimental group comparing with that of the control group,while there was no statistically significant difference in the 6-month mortality rate between the groups within the CST-intact subgroup(P=1.000).There were no statistically significant differences between the experimental group and the control group in both efficacy and safety indices within the CST-damaged subgroup(all P>0.05).Conclusions Infarct core resection combining DC with CST protection demonstrates superior neurological functional improvement in comparison with anterior temporal lobectomy combining DC in treating MCI with malignant brain edema,particularly for patients with an intact CST before surgery(as indicated in patients'preoperative imaging results).This(infarct core resection combining DC with CST protection)approach does not increase the incidence of surgical complications.Prospective large sample controlled studies are required for further validation.
8.Clinical efficacy of internal decompression based on white matter tract preservation in treatment of malignant middle cerebral artery infarction
Yanli ZHANG ; Menglu LI ; Jiankai ZHAO ; Jingmin DOU ; Fei ZHANG ; Baoming JIA ; Guoqiang FENG ; Hongbin KU
Chinese Journal of Nervous and Mental Diseases 2025;51(10):596-600
Objective To investigate the clinical efficacy of internal decompression based on white matter tract preservation in the treatment of malignant middle cerebral artery infarction(MMCAI).Methods A retrospective analysis was conducted on 54 patients with MMCAI.Patients were divided into a study group(n=26)and a control group(n=28)according to the surgical approach.Patients in the study group underwent preoperative fusion of CT,CTP,DWI,and DTI imaging data within a neuronavigation system.This fusion visualized the spatial relationships between the infarct core(IC),ischemic penumbra,and the corticospinal tract(CST).Subsequently,IC resection combined with decompressive craniectomy(DC)was performed while protecting the CST.Patients in the control group underwent DC alone.Key outcome measures included:changes in fractional anisotropy(FA)within the affected CST projection area at 1 month postoperatively;and 6-month postoperative mRS score,mortality,and surgical complications at 6 months postoperatively.Results At 1 month postoperatively,FA in the affected CST projection area were significantly higher in the study group than in the control group(0.092±0.013 vs.0.082±0.008,P<0.05).At the 6-month follow-up,the postoperative mRS score in the study group was significantly lower than that in the control group[2.3(1.3,4.5)vs.3.9(2.4,5.5),P<0.05]and a lower mortality rate(11.5%vs.39.3%,P<0.05)compared to the control group.However,there were no statistically significant differences between the two groups in the incidence of postoperative intracranial hemorrhage,intracranial infection,or epilepsy(P>0.05).Conclusion Internal decompression based on white matter tract protection combined with DC can reduce mortality and contribute to improving function outcomes in patients with MMCAI.
9.Correlation Between Fractional Area Change of Left Ventricle Measured by Cardiac Magnetic Resonance and Myocardial Circumferential Strain
Menglu LI ; Heng ZHANG ; Chunjie WANG ; Fuzhuang JIANG ; Kaizhou YANG ; Yuanyuan XING ; Fangmin PEI ; Liuquan CHENG
Chinese Journal of Medical Imaging 2025;33(7):739-744
Purpose To compare the similarity between the short-axis left ventricular cavity fractional area change measured by cardiac magnetic resonance and myocardial circumferential strain.Materials and Methods Forty cases of cardiomyopathy with different phenotypes and high-quality cardiac magnetic resonance images in PACS data center of the Sixth Medical Center of Chinese PLA General Hospital were retrospectively selected.All patients underwent cine imaging on a 3.0T MRI scanner.The endocardial and epicardial areas of each short-axis left ventricular slice were measured using Medviso Segment software to calculate slice-level fractional area change and global fractional area change.Slice circumferential strain and global circumferential strain were measured using Circle CVI42 software.Linear regression analysis was performed to assess correlations between global circumferential strain and global fractional area change,as well as slice circumferential strain and slice-level fractional area change.Results Both global circumferential strain and global fractional area change,as well as slice circumferential strain and slice-level fractional area change,exhibited positive correlations(all r>0.95).Linear regression demonstrated significant relationships(regression coefficients:2.40-3.16,P<0.05),with coefficient magnitudes related to left ventricular cavity radius.After normalization,circumferential strain and fractional area change curves showed identical standard deviations.Conclusion Short-axis left ventricular cavity fractional area change and myocardial circumferential strain display significant positive correlations at both slice and global levels,with similar curve morphology.These findings suggest that circumferential strain and fractional area change possess comparable statistical value in assessing cardiac function.
10.Correlation between complement deposition levels on blood cells and clinical biomarkers in patients with paroxysmal nocturnal hemoglobinuria
Menglu ZHANG ; Xuan WANG ; Chen YANG ; Miao CHEN ; Bing HAN
Chinese Journal of Hematology 2025;46(4):314-320
Objective:To explore the complement deposition levels on blood cell surfaces in patients with paroxysmal nocturnal hemoglobinuria (PNH) and evaluate their association with clinical manifestations.Methods:This study enrolled patients with PNH, who had not been treated with complement inhibitors and appeared at Peking Union Medical College Hospital from February 2021 to February 2023. The clinical information of participants was retrospectively recorded, and peripheral blood samples were collected. Gender- and age-matched normal controls (NC) were recruited accordingly. C5b-9, C3, C4b, and factor B (FB) deposition levels on peripheral red blood cells, white blood cells, and platelets were detected with flow cytometry. The correlation between complement deposition levels and clinical symptoms was analyzed.Results:This study involved 73 patients with PNH, including 42 (57.5%) males, with a median age of 36 (range: 14-76) years. 16 matched NC were collected. Among patients with PNH, 36 (49.3%) had classical PNH and 37 (50.7%) had aplastic anemia-PNH syndrome. Thromboembolic events (TEE) occurred in 18 (24.7%) patients. The median HGB, absolute reticulocyte count (Ret), and lactate dehydrogenase of PNH patients were 76 (37-116) g/L, 181.0 (45.9-495.8) ×10 9/L, and 1 875 (377 - 5 509) U/L, respectively. The median number of Flaer-negative white blood cells was 94.0% (13.0% - 99.9%) ; the median CD59 negative red blood cells was 46.7% (9.0% - 93.0%). The deposition of C5b-9, C3, C4b, and FB on red blood cells, white blood cells, and platelets in patients with PNH was significantly higher than that in NC (all P<0.05). C5b-9 deposition level was significantly higher than that of C3, C4b, and FB on all three blood cell lineages in PNH patients (all P<0.01). The deposition of all complement fragments on red blood cells was significantly lower than that on white blood cells and platelets (all P<0.01). C5b-9 deposition on red blood cells was positively correlated with Ret in PNH patients ( P=0.005). C3 ( P=0.001) and C4b ( P=0.017) deposition levels on white blood cells and C3 deposition on platelets ( P=0.002) in patients with TEE history were lower than those without. Conclusions:C5b-9, C3, C4b, and FB deposition levels on all three blood cells in patients with PNH were higher than NC. Increased C5b-9 on red blood cells may indicate active hemolysis. Reduced C3 and C4b levels on white blood cells and low C3 deposition on platelets may indicate TEE risk.

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