Objective: To conduct screening for rare blood types within important blood group systems for the Chinese population, such as Rh, Duffy, Kidd, P1Pk, Diego, and MNS, in the Guangzhou region, and to establish a corresponding rare blood type database and physical repository. Methods: The saline medium microplate method was used to screen blood donors with the ccDEE phenotype combined with either Jk(a-) or Jk(b-). The polybrene microplate method was employed to screen for donors with Fy(a-), s(-), Lu(b-), Di(b-), k(-), and p phenotypes. The urea lysis microplate method was applied to screen for the Jk(a-b-) phenotype. A high-resolution melting (HRM) curve method was established for screening some donors with the Di(b-) phenotype. Subsequently, expanded phenotyping of antigens in the Rh, Kidd, MNS, Duffy, P1Pk, Lewis, Kell, and Lutheran blood group systems was performed on identified rare blood type donors using monoclonal antibodies. The test results are entered into the Rare Blood Type Bank Management System of the Guangzhou Blood Center, enabling functions such as confirmation reminders and cryopreservation storage when the donor donates again. Red blood cells of rare blood types are processed into frozen red blood cells for long-term storage. Results: Among voluntary blood donors, 16 cases of the ccDEE combined with Jk(a-) phenotype were identified (0.221 7%, 16/7 216); 10 cases of the ccDEE combined with Jk(b-) phenotype (0.138 6%, 10/7 216); 78 cases of the Fy(a-) phenotype (0.169 5%, 78/46 012); 39 cases of the Lu(b-) phenotype (0.138 2%, 39/28 214); 31 cases of the s(-) phenotype (0.081 8%, 31/37 913); 22 cases of the Di(b-) phenotype (0.029 9%, 22/73 691); 30 cases of the Jk(a-b-) phenotype (0.010 1%, 30/298 250); and 1 case of the k(-) phenotype (0.001 3%, 1/77 382), which was further identified as KELnull phenotype (K0). No p phenotype donors were identified (0/88 528). A total of 228 units of frozen red blood cells were prepared. The screening results were compared and analyzed with rare blood type data from other regions. Conclusion: This study, through a combination of different screening methods, significantly improved the efficiency of rare blood type screening while remaining cost-effective. By conducting large-scale screening and performing data informatization processing, a database and physical repository of rare blood types in the Guangzhou region were successfully established. This provides a strong guarantee for the timely supply of blood to patients with difficult-to-match and rare blood types in the region, effectively enhances the level of transfusion safety in the region, and offers a practical paradigm for constructing a comprehensive blood transfusion support system.

OBJECTIVE To establish a multi-index quantitative detection method,and to evaluate the quality difference of Gleditsia sinensis from different producing areas.METHODS The contents of protocatechuic acid,vanillic acid,isoscopoletin,scoparone,isovitexin,fustin,taxifolin,fisetin,quercetin,kaempferol,echinocystic acid,betulinic acid,β-sitosterol and stigmasterol were detected by high performance liquid chromatography-quantitative analysis of multi-components by single marker(HPLC-QAMS).The chromatographic column was Kromasil C18,the mobile phase was 0.2％phosphoric acid-acetonitrile solution(gradient elution),the detection wavelengths were 254,360,210 nm for different index components,the column temperature was 30 ℃,the flow rate was 1.0 mL/min,and the sample injection volume was 10 μL.The contents of extract and total ash were detected according to the method of Chinese Pharmacopoeia.The quality differences of 30 batches of G.sinensis(No.S1-S30)from different producing areas were evaluated by chemometrics,weighted technique for order preference by similarity to an ideal solution(TOPSIS)analysis and Logistic regression model.RESULTS The linear ranges of 14 components were 1.55-77.50,0.71-35.50,0.28-14.00,0.96-48.00,1.77-88.50,0.09-4.50,4.65-232.50,1.49-74.50,0.37-18.50,1.18-59.00,7.35-367.50,3.58-179.00,0.49-24.50 and 0.21-10.50 μg/mL,respectively(all r＞0.999).The RSDs of precision,stability(24 h)and repeatability were less than 2.00％;the average recoveries were 96.99％-100.13％(all RSDs＜2.00％),and the relative correction factor had good repeatability.The contents of extract and total ash were 4.2％-12.5％and 0.5％-2.3％,respectively.There was no significant difference in the content of 14 components measured by QAMS method and extemal standard method(P＞0.05).The results of chemometrics showed that 30 batches of samples were clustered into 3 categories:S1 to S11 form one category,S12 to S20 form another category,and S21 to S30 constitute the third category.Echinocystic acid,betulinic acid,taxifolin,kaempferol,isovitexin,scoparone and protocatechuic acid may be the differential components affecting the quality of G.sinensis from different producing areas.The analysis results of the weighted TOPSIS method revealed that relative closeness(Jb)for 30 batches of G.sinensis ranged from 0.144 5 to 0.721 8,with S27 achieving the highest value(Jb)of 0.721 8.The analysis results of the Logistic regression model showed that S21-S30 batches of samples were of superior grade,S1-S11 were of intermediate grade,and S12-S20 were of inferior grade.CONCLUSIONS The established HPLC-QAMS method is simple and accurate.The comprehensive evaluation method is objective and comprehensive,and can be used to evaluate the quality difference of G.sinensis from different producing areas.

Objective To establish a method for quantitative control of multi-pharmacological components in Kanggan Jiedu capsules,and to evaluate the quality of Kanggan Jiedu capsules by chemometrics and weighted technique for order preference by similarity to an ideal solution(TOPSIS)method.Methods HPLC was performed on an Apollo C18 column with acetonitrile-0.1%formic acid gradient elution as mobile phase.The volume flow rate was 1.0 mL/min,and the detection wavelength was 327,238 and 298 nm.The contents of 3,5-O-dicaffeoylquinic acid,4,5-dicaffeoylquinic acid,gardenoside,geniposide,3'-hydroxy puerarin,puerarin,3'-methoxy puerarin,baicalin,wogonoside,oxypeucedanin,imperatorin,isoimperatorin,pseudoaspidin and tetraalbaspidin ABBA in 15 batches of Kanggan Jiedu capsules were determined by quantitative analysis of multi-components by single marker(QAMS),and then statistical software was used to analyze the difference of 15 batches of samples to find out the main potential markers affecting the quality of Kanggan Jiedu capsules.Results The f value of the 3,5-O-dicaffeoylquinic acid was 0.9119,with 4,5-dicaffeoylquinic acid as the internal reference.The f of the gardenoside,3'-hydroxy puerarin,puerarin and 3'-methoxy puerarin were 1.4941,1.2423,0.9281 and 1.0860,respectively,with geniposide as the internal reference.The f of the baicalin,wogonoside,oxypeucedanin,isoimperatorin,pseudoaspidin and tetraalbaspidin ABBA were 1.4551,0.7414,0.8796,0.6949,1.3248 and 0.8240,respectively,with imperatorin as the internal reference.The f values of each component were robust.There was no significant difference in the content determined by the two methods.The chemometrics method results showed that 15 batches of Kanggan Jiedu capsules could be clustered into three categories,there were certain differences between batches.Baicalin,geniposide,imperatorin,puerarin,3,5-O-dicaffeoylquinic acid and wogonoside were the main potential markers affecting the quality of Kanggan Jiedu capsules.The results of weighted TOPSIS method results showed that the Ci values of 15 batches of samples were between 0.2018 and 0.7346,among which S13 ranked the top with Ci value of 0.7346.Conclusion The established method for quantitative detection of 14 pharmacodynamic components is convenient and accurate.The quality of Kanggan Jiedu capsules can be evaluated by chemometrics and weighted TOPSIS method.

Objective: To investigate the impact of RHAG 808A variant, commonly identified in the Chinese population, on RhAG protein, RhD and RhCE antigens expression through in vivo and in vitro expression analysis. Methods: A missense mutation of RHAG gene (c. 808G>A, p. Val270Ile) with high frequency was found in KMxD database. Bioinformatics analysis was performed using Polyphen-2 and Provean software. High resolution melting (HRM) method was utilized to screen for the variant carriers in the blood donors. The expression of RhAG protein, RhD and RhCE antigens on the surface of red cells of variant carriers were detected via flow cytometry. Wild-type and mutant vectors of RHAG were constructed and transfected into HEK 293T cells for in vitro expression analysis. Then, the expression of RhAG protein, RhD and RhCE antigens were analyzed by flow cytometry. Results: Polyphen-2 and Provean software suggested that the amino acid change (p. Val270Ile) of RhAG protein may be harmful or neutral respectively. Among the 999 blood donors from Guangzhou Blood Center, 4 homozygous carriers and 99 heterozygous carriers of RHAG 808A mutant allele were identified. The frequency of this allele was 5.4% (107/1 998). No significant differences in RhAG protein, RhD and RhCE antigens expression level was identified between the homozygous carriers, heterozygous carriers of RHAG 808A variant allele and the wild-type individuals. In vitro analysis for antigen expression study obtained the similar results. Conclusion: The RHAG 808A variant allele commonly identified in the Chinese population has no effect on the expression of RhAG protein, RhD and RhCE antigens, so the variant should be a population polymorphism site.

Objective:To introduce a new technique of precise exposure and preservation of membranous semicircular canal under endoscope, and to evaluate the protective effect of this method on hearing preservation of semicircular canal occlusion.Methods:The study included three patients with unilateral refractory Meniere′s disease, including 1 male and 2 females, with hearing stage 4 in 2 cases and stage 3 in 1 case, the average hearing thresholds were 72.5, 82.5 and 48.8 dBHL, respectively. All of them were treated with triple semicircular canal occlusion under continuous irrigating mode of endoscopic ear surgery (CIM-EES) in RenMin Hospital of WuHan University. The surgical procedure involved the following key steps: the bony walls of the three semicircular canals near the ampullae were gradually thinned to expose the blue line. The endoscope was positioned as close as possible to the operating area to obtain a clear, magnified view. A micro hook needle was used to create a window on the bony semicircular canals. The hook needle progressively removed the bony material while preserving the membranous canals, which were clearly visible during the procedure. The bone dust was removed out of the surgical area by the continuous exchanged insuline without the help of suction that can avoid the damage of the membranous semicircular canals caused by aspiration and the open bony canals were precisely filled with bone wax and covered with bone powder to ensure complete occlusion of the three semicircular canals. Postoperative follow-up included regular assessments of vertigo control and hearing preservation.Results:All the 3 patients obtained complete hearing preservation at 6 months after surgery, and the hearing preservation rate was 100%. The average hearing thresholds at 6 months postoperatively were 66.3, 81.3, and 50.0 dBHL, respectively. Postoperatively, all patients experienced horizontal spontaneous nystagmus toward the healthy side on the day of surgery and the first day after surgery. One patient reported complete disappearance of tinnitus, while the other two patients showed no change in tinnitus severity. Postoperative MRI revealed complete occlusion of the three semicircular canals with normal vestibular imaging. At 6 months postoperatively, none of the patients experienced any vertigo attacks, suggesting effective control of vertigo symptoms.Conclusions:Precise Semicircular canal occlusion under CIM-EES is a promising technique for the treatment of intractable vertigo in patients with refractory Meniere′s disease. This method provides clear visualization of the membranous canals, reducing the risk of inner ear damage and preserving hearing function of the patients.

Objective:To investigate the clinical characteristics of adult patients with autoimmune disease-associated hemophagocytic syndrome (AID-HPS) and enhance clinical recognition of this condition.Methods:A retrospective analysis was performed on 85 adult AID-HPS patients admitted to the department of rheumatology and immunology at Peking Union Medical College Hospital from January 2012 to December 2020. Clinical data included demographics, predisposing factors, manifestations, laboratory/imaging findings, treatments, and outcomes. Patients were stratified into three groups based on underlying AIDs: adult-onset Still′s disease with HPS (AOSD-HPS) group, systemic lupus erythematosus with HPS (SLE-HPS) group, and other AID with HPS (other AID-HPS) group. Comparative analyses were conducted to identify intergroup differences. Continuous variables were analyzed using one-way ANOVA, Welch′s test, or Kruskal-Wallis′s test based on data distribution and homogeneity of variance. Categorical variables (rates and proportions) were compared with the chi-square test or Fisher-Freeman-Halton exact test according to expected cell frequencies.Results:Among 85 patients, 67 were female. Underlying AIDs included AOSD (32 cases, 37.6%), SLE (32 cases, 37.6%), and other AIDs (21 cases, 24.7%). Infections (≥1 type) were identified in 54 patients (63.5%), predominantly viral (48 cases, 56.5%), including cytomegalovirus (CMV) (40 cases), Epstein-Barr virus (EBV) (11 cases), and 6 cases were coinfected with both CMV and EBV. All patients presented with fever; hepatomegaly, splenomegaly, and lymphadenopathy were observed in 39 (45.9%), 59 (69.4%), and 70 (82.4%) cases, respectively. Additional manifestations included arthralgia (63 cases, 74.1%) and rash (65 cases, 76.5%). Pancytopenia in 31 cases (36.5%) and bicytopenia in 29 cases (34.1%). Liver dysfunction was noted in 79 cases (92.9%). Elevated lactate dehydrogenase (LDH) (83 cases, 97.6%), elevated triglyceride (TG) (76 cases, 89.4%), decreased fibrinogen (Fbg) (55 cases, 64.7%), and elevated serum ferritin (SF) (84 cases, 98.8%) were common. Elevated soluble CD25(sCD25) (53cases) and reduced NK cell activity (49 cases) were observed. Bone marrow examination revealed hemophagocytosis in 49 cases. There were statistically significant differences in age( F=3.763, P=0.031), lymphadenopathy( χ2=7.098, P=0.029), rash( χ2=12.816, P=0.002), reductions in WBC( H=22.284, P<0.001)、NEU( H=18.882, P<0.001)、PLT( H=15.127, P=0.001), and elevations in LDH( H=7.842; P=0.020)、TG( H=6.177, P=0.046)、CRP( H=6.915, P=0.032)、SF( H=9.661, P=0.008)、sCD25( χ2=5.154, P=0.035) among the three groups: (1) The SLE-HPS group was significantly younger [(28.1 ± 10.4) years) than the other AID-HPS group [(39.5 ± 17.3) years, P=0.028]. (2) The AOSD-HPS group had higher incidence rates of lymphadenopathy (93.8%) and rash (93.8%) than the SLE-HPS group (68.8% and 56.3%, respectively), (lymphadenopathy: χ2=7.098, P=0.029; rash: χ2=12.816, P=0.002). (3) ① WBC in the SLE-HPS group [1.62 (1.18, 2.92) ×10 9/L] were significantly lower than those in the AOSD-HPS group [5.66 (2.75, 11.57)×10 9/L] and the other AID-HPS group [6.05 (2.49, 14.55)×10 9/L] ( Z=-4.032, P<0.001; Z=3.993, P<0.001). ② NEU in the SLE-HPS group [1.10 (0.60, 1.93)×10 9/L] were markedly reduced compared to the AOSD-HPS group [3.73 (1.54, 9.04)×10 9/L] and the other AID-HPS group [2.23 (1.43, 11.57)×10 9/L] ( Z=-3.859, P<0.001; Z=3.506, P=0.001). ③ PLT in the SLE-HPS group [59.50 (28.50, 81.00)×10 9/L] were significantly lower than those in the AOSD-HPS group [109.00 (65.75, 232.00)×10 9/L] and the other AID-HPS group [150.00 (55.00, 221.00)×10 9/L] ( Z=-3.421, P=0.002; Z=3.179, P=0.004). (4) LDH levels in the AOSD-HPS group [1 178 (645, 1 875) U/L] were significantly higher than those in the other AID-HPS group [598 (410, 771) U/L] ( Z=2.795, P=0.016). (5) TG levels in the SLE-HPS group [3.61 (2.46, 6.09) mmol/L] were significantly higher than those in the other AID-HPS group [2.68 (1.71, 3.30)mmol/L] ( Z=2.402, P=0.049). (6) CRP and SF levels in the AOSD-HPS group [79.20 (28.02, 179.53)mg/L and 30 225 (13 494, 53 598)μg/L, respectively] were significantly higher than those in the SLE-HPS group [26.05 (9.41, 83.31)mg/L and 9 862 (4 467, 22 315) μg/L, respectively] ( Z=2.547, P=0.033; Z=3.069, P=0.006 ). (7) The incidence rates with elevated sCD25 in the AOSD-HPS group (100.0%) was significantly higher than that in the other AID-HPS group (76.9%), ( χ2=5.154, P=0.035). After treatment, 83 patients improved, while 2 deaths occurred in the other AID-HPS group. Conclusion:Adult AID-HPS predominantly affects young to middle-aged females, with SLE and AOSD being the most common underlying AIDs. The condition manifests with severe clinical features, frequently triggered by viral infections (particularly CMV and EBV). Distinct differences in clinical and laboratory profiles exist among AID-HPS subtypes. Early recognition and aggressive treatment are critical for improving prognosis.

Objective To establish a method for quantitative control of multi-pharmacological components in Kanggan Jiedu capsules,and to evaluate the quality of Kanggan Jiedu capsules by chemometrics and weighted technique for order preference by similarity to an ideal solution(TOPSIS)method.Methods HPLC was performed on an Apollo C18 column with acetonitrile-0.1%formic acid gradient elution as mobile phase.The volume flow rate was 1.0 mL/min,and the detection wavelength was 327,238 and 298 nm.The contents of 3,5-O-dicaffeoylquinic acid,4,5-dicaffeoylquinic acid,gardenoside,geniposide,3'-hydroxy puerarin,puerarin,3'-methoxy puerarin,baicalin,wogonoside,oxypeucedanin,imperatorin,isoimperatorin,pseudoaspidin and tetraalbaspidin ABBA in 15 batches of Kanggan Jiedu capsules were determined by quantitative analysis of multi-components by single marker(QAMS),and then statistical software was used to analyze the difference of 15 batches of samples to find out the main potential markers affecting the quality of Kanggan Jiedu capsules.Results The f value of the 3,5-O-dicaffeoylquinic acid was 0.9119,with 4,5-dicaffeoylquinic acid as the internal reference.The f of the gardenoside,3'-hydroxy puerarin,puerarin and 3'-methoxy puerarin were 1.4941,1.2423,0.9281 and 1.0860,respectively,with geniposide as the internal reference.The f of the baicalin,wogonoside,oxypeucedanin,isoimperatorin,pseudoaspidin and tetraalbaspidin ABBA were 1.4551,0.7414,0.8796,0.6949,1.3248 and 0.8240,respectively,with imperatorin as the internal reference.The f values of each component were robust.There was no significant difference in the content determined by the two methods.The chemometrics method results showed that 15 batches of Kanggan Jiedu capsules could be clustered into three categories,there were certain differences between batches.Baicalin,geniposide,imperatorin,puerarin,3,5-O-dicaffeoylquinic acid and wogonoside were the main potential markers affecting the quality of Kanggan Jiedu capsules.The results of weighted TOPSIS method results showed that the Ci values of 15 batches of samples were between 0.2018 and 0.7346,among which S13 ranked the top with Ci value of 0.7346.Conclusion The established method for quantitative detection of 14 pharmacodynamic components is convenient and accurate.The quality of Kanggan Jiedu capsules can be evaluated by chemometrics and weighted TOPSIS method.

Objective:To introduce a new technique of precise exposure and preservation of membranous semicircular canal under endoscope, and to evaluate the protective effect of this method on hearing preservation of semicircular canal occlusion.Methods:The study included three patients with unilateral refractory Meniere′s disease, including 1 male and 2 females, with hearing stage 4 in 2 cases and stage 3 in 1 case, the average hearing thresholds were 72.5, 82.5 and 48.8 dBHL, respectively. All of them were treated with triple semicircular canal occlusion under continuous irrigating mode of endoscopic ear surgery (CIM-EES) in RenMin Hospital of WuHan University. The surgical procedure involved the following key steps: the bony walls of the three semicircular canals near the ampullae were gradually thinned to expose the blue line. The endoscope was positioned as close as possible to the operating area to obtain a clear, magnified view. A micro hook needle was used to create a window on the bony semicircular canals. The hook needle progressively removed the bony material while preserving the membranous canals, which were clearly visible during the procedure. The bone dust was removed out of the surgical area by the continuous exchanged insuline without the help of suction that can avoid the damage of the membranous semicircular canals caused by aspiration and the open bony canals were precisely filled with bone wax and covered with bone powder to ensure complete occlusion of the three semicircular canals. Postoperative follow-up included regular assessments of vertigo control and hearing preservation.Results:All the 3 patients obtained complete hearing preservation at 6 months after surgery, and the hearing preservation rate was 100%. The average hearing thresholds at 6 months postoperatively were 66.3, 81.3, and 50.0 dBHL, respectively. Postoperatively, all patients experienced horizontal spontaneous nystagmus toward the healthy side on the day of surgery and the first day after surgery. One patient reported complete disappearance of tinnitus, while the other two patients showed no change in tinnitus severity. Postoperative MRI revealed complete occlusion of the three semicircular canals with normal vestibular imaging. At 6 months postoperatively, none of the patients experienced any vertigo attacks, suggesting effective control of vertigo symptoms.Conclusions:Precise Semicircular canal occlusion under CIM-EES is a promising technique for the treatment of intractable vertigo in patients with refractory Meniere′s disease. This method provides clear visualization of the membranous canals, reducing the risk of inner ear damage and preserving hearing function of the patients.

Objective:To investigate the clinical characteristics of adult patients with autoimmune disease-associated hemophagocytic syndrome (AID-HPS) and enhance clinical recognition of this condition.Methods:A retrospective analysis was performed on 85 adult AID-HPS patients admitted to the department of rheumatology and immunology at Peking Union Medical College Hospital from January 2012 to December 2020. Clinical data included demographics, predisposing factors, manifestations, laboratory/imaging findings, treatments, and outcomes. Patients were stratified into three groups based on underlying AIDs: adult-onset Still′s disease with HPS (AOSD-HPS) group, systemic lupus erythematosus with HPS (SLE-HPS) group, and other AID with HPS (other AID-HPS) group. Comparative analyses were conducted to identify intergroup differences. Continuous variables were analyzed using one-way ANOVA, Welch′s test, or Kruskal-Wallis′s test based on data distribution and homogeneity of variance. Categorical variables (rates and proportions) were compared with the chi-square test or Fisher-Freeman-Halton exact test according to expected cell frequencies.Results:Among 85 patients, 67 were female. Underlying AIDs included AOSD (32 cases, 37.6%), SLE (32 cases, 37.6%), and other AIDs (21 cases, 24.7%). Infections (≥1 type) were identified in 54 patients (63.5%), predominantly viral (48 cases, 56.5%), including cytomegalovirus (CMV) (40 cases), Epstein-Barr virus (EBV) (11 cases), and 6 cases were coinfected with both CMV and EBV. All patients presented with fever; hepatomegaly, splenomegaly, and lymphadenopathy were observed in 39 (45.9%), 59 (69.4%), and 70 (82.4%) cases, respectively. Additional manifestations included arthralgia (63 cases, 74.1%) and rash (65 cases, 76.5%). Pancytopenia in 31 cases (36.5%) and bicytopenia in 29 cases (34.1%). Liver dysfunction was noted in 79 cases (92.9%). Elevated lactate dehydrogenase (LDH) (83 cases, 97.6%), elevated triglyceride (TG) (76 cases, 89.4%), decreased fibrinogen (Fbg) (55 cases, 64.7%), and elevated serum ferritin (SF) (84 cases, 98.8%) were common. Elevated soluble CD25(sCD25) (53cases) and reduced NK cell activity (49 cases) were observed. Bone marrow examination revealed hemophagocytosis in 49 cases. There were statistically significant differences in age( F=3.763, P=0.031), lymphadenopathy( χ2=7.098, P=0.029), rash( χ2=12.816, P=0.002), reductions in WBC( H=22.284, P<0.001)、NEU( H=18.882, P<0.001)、PLT( H=15.127, P=0.001), and elevations in LDH( H=7.842; P=0.020)、TG( H=6.177, P=0.046)、CRP( H=6.915, P=0.032)、SF( H=9.661, P=0.008)、sCD25( χ2=5.154, P=0.035) among the three groups: (1) The SLE-HPS group was significantly younger [(28.1 ± 10.4) years) than the other AID-HPS group [(39.5 ± 17.3) years, P=0.028]. (2) The AOSD-HPS group had higher incidence rates of lymphadenopathy (93.8%) and rash (93.8%) than the SLE-HPS group (68.8% and 56.3%, respectively), (lymphadenopathy: χ2=7.098, P=0.029; rash: χ2=12.816, P=0.002). (3) ① WBC in the SLE-HPS group [1.62 (1.18, 2.92) ×10 9/L] were significantly lower than those in the AOSD-HPS group [5.66 (2.75, 11.57)×10 9/L] and the other AID-HPS group [6.05 (2.49, 14.55)×10 9/L] ( Z=-4.032, P<0.001; Z=3.993, P<0.001). ② NEU in the SLE-HPS group [1.10 (0.60, 1.93)×10 9/L] were markedly reduced compared to the AOSD-HPS group [3.73 (1.54, 9.04)×10 9/L] and the other AID-HPS group [2.23 (1.43, 11.57)×10 9/L] ( Z=-3.859, P<0.001; Z=3.506, P=0.001). ③ PLT in the SLE-HPS group [59.50 (28.50, 81.00)×10 9/L] were significantly lower than those in the AOSD-HPS group [109.00 (65.75, 232.00)×10 9/L] and the other AID-HPS group [150.00 (55.00, 221.00)×10 9/L] ( Z=-3.421, P=0.002; Z=3.179, P=0.004). (4) LDH levels in the AOSD-HPS group [1 178 (645, 1 875) U/L] were significantly higher than those in the other AID-HPS group [598 (410, 771) U/L] ( Z=2.795, P=0.016). (5) TG levels in the SLE-HPS group [3.61 (2.46, 6.09) mmol/L] were significantly higher than those in the other AID-HPS group [2.68 (1.71, 3.30)mmol/L] ( Z=2.402, P=0.049). (6) CRP and SF levels in the AOSD-HPS group [79.20 (28.02, 179.53)mg/L and 30 225 (13 494, 53 598)μg/L, respectively] were significantly higher than those in the SLE-HPS group [26.05 (9.41, 83.31)mg/L and 9 862 (4 467, 22 315) μg/L, respectively] ( Z=2.547, P=0.033; Z=3.069, P=0.006 ). (7) The incidence rates with elevated sCD25 in the AOSD-HPS group (100.0%) was significantly higher than that in the other AID-HPS group (76.9%), ( χ2=5.154, P=0.035). After treatment, 83 patients improved, while 2 deaths occurred in the other AID-HPS group. Conclusion:Adult AID-HPS predominantly affects young to middle-aged females, with SLE and AOSD being the most common underlying AIDs. The condition manifests with severe clinical features, frequently triggered by viral infections (particularly CMV and EBV). Distinct differences in clinical and laboratory profiles exist among AID-HPS subtypes. Early recognition and aggressive treatment are critical for improving prognosis.

【Objective】 To identify the specificity of alloantibody against high-frequency antigens in one case suffering with severe hemolytic diseases of the fetus and newborn (HDFN) and to screen for matching blood for transfusion. 【Methods】 The HDFN test and the antibody serological identification tests in the mother were performed. Several common high frequency antigens of maternal red blood cells (RBCs) were determined. IgG subtype coated on the RBCs of the newborn was determined. The phagocytic efficiency of the antibody was tested using the monocyte phagocytosis of sensitized erythrocyte by flow cytometry in vitro. Sanger sequencing of DI gene was performed in the mother, father and mother’s brother. The diluted maternal plasma was used for large scale screening of matching blood using IAT in Coomb’s gel card. 【Results】 Di(b-) phenotype was identified in the mother of the newborn and anti-Di^b (titer: 512) related HDN was detected in the newborn. IgG1 and IgG2 subtypes of anti-Di^b were detected and the rate of monocyte phagocytosis was 88.83%(74.7/84.09). The compatible blood was not detected in the maternal relatives. Subsequently, the newborn received the matching RBCs of two Di(b-) donors identified from 5 520 blood donors and discharged from the hospital. We screened out 17 Di(b-) donors out of 51 334 blood donors, indicating that the distribution frequency of Di(b-) among blood donors in Guangzhou was about 0.033% (17/51 334). 【Conclusion】 By serology and molecular biology methods, the newborn was identified with HDFN caused by anti-Di^b, and an effective large-scale screening method for Di (b -) rare blood types was established to find matching blood, which supported the establishment of rare Di(b-) blood database.