Objective:To evaluate the impact of donor characteristics on the prognosis of myelodysplastic syndrome (MDS) patients undergoing haplo-identical transplantation (HIDT) .Methods:A retrospective analysis of 203 MDS patients who received HIDT was conducted to evaluate how donor factors influenced transplant outcomes.Results:In MDS patients undergoing haploidentical transplantation, donors over 50 years were associated with higher EBV reactivation (2-year cumulative incidence 42.9% vs 22.0% for <50 years old; P=0.010). Female donors were linked to increased severe chronic GVHD compared with male donors (2-year incidence 11.9% vs 4.0% ; P=0.017). Additionally, 2-year overall survival (OS) was slightly lower with female donors than male donors (56.6% vs 69.7% ), but the difference was not statistically significant ( P=0.073). Donor-recipient blood type did not affect post-transplant OS or cumulative relapse rates. Donor-recipient kinship analysis revealed that child donors, compared to haploidentical sibling or parent donors, had lower rates of grade Ⅱ–Ⅳ acute GVHD (27.2% vs 45.7% vs 53.5%, P=0.007) and 2-year EBV reactivation (13.9% vs 29.3% vs 38.9%, P=0.001). For donors under 20 years, donor gender did not significantly affect 2-year OS ( P=0.913), relapse-free survival ( P=0.716), or 100-day incidence of grade Ⅱ–Ⅳ acute GVHD ( P=0.359) . Conclusion:For MDS patients undergoing HIDT, donors over 50 should be avoided. Male and child donors are preferred, while donor gender does not significantly affect outcomes if the donor is under 20 years old.

Objective:To establish a flow cytometric bead assay (FCBA) for detecting von Willebrand factor (vWF) activity based on mutant GPⅠbα and to perform its clinical validation.Methods:Recombinant GPⅠbα protein with M239V, D235Y, and C65A point mutations was constructed and bound to flow cytometric beads. vWF in samples bound to the recombinant protein, and FITC-labeled rabbit anti-human vWF polyclonal antibody was added for detection and analysis using flow cytometry. A total of 32 patients with von Willebrand disease (10 males and 22 females, aged 26.81±6.96 years) admitted to the Department of Hematology at the First Affiliated Hospital of Soochow University from January 1, 2022, to November 30, 2023, were enrolled. Additionally, 51 healthy controls (17 males and 34 females, aged 31.56±8.98 years) were included. Plasma from 20 healthy controls was pooled in equal proportions to create standard plasma. Fourteen dilutions were prepared, ranging from the original plasma to a 1∶8, 192 dilution, and a curve was plotted according to the FCBA protocol to determine the optimal dilution. Plasma from one healthy control was aliquoted and frozen, and FCBA was performed on days 1, 5, 15, 30, 45, and 60 after bead coating to evaluate stability. The same healthy control plasma sample was tested 20 times using both FCBA and vWF enzyme-linked immunesorbent assay (ELISA) to calculate within-batch precision. The same sample was tested daily for 10 consecutive days using both FCBA and vWF-ELISA to calculate between-batch precision. All samples were tested using FCBA, and the same samples were also tested using vWF-ELISA. The χ2 test was used to compare the positive rates, negative rates, and accuracy of the two methods. One sample was used as the base sample, and another sample with a known activity of 100% was diluted to high, medium, and low activities and added to the base sample. Recovery rates were calculated using both FCBA and vWF-ELISA. Re-collected 11 cases of vWD patient samples and 10 cases of healthy subjects, and detected vWF activity using the FCBA method to validate the concordance of FCBA in clinical applications. Results:The optimal dilution for standard plasma was determined to be 1∶64. The coated beads remained stable for detection up to 60 days, with correlation coefficients of the standard curve on days 1, 5, 15, 30, 45, and 60 being 0.98, 0.98, 0.97, 0.95, 0.95, and 0.95, respectively. The within-batch coefficients of variation for FCBA and vWF-ELISA were 5.35% and 6.08%, respectively, while the between-batch coefficients of variation were 7.02% and 7.98%, respectively. The correlation coefficient between FCBA and vWF-ELISA was R2=0.798 ( P0.01). The positive rates were 90.63% and 84.38% ( χ2=0.571, P0.05), the negative rates were 92.16% and 94.12% ( χ2=0.153, P0.05), and the accuracy rates were 91.57% and 90.36% ( χ2=0.073, P0.05) for FCBA and vWF-ELISA, respectively. In the recovery experiment, the high-value recovery rates for FCBA and ELISA were 102.11% and 99.32%, respectively, the medium-value recovery rates were 98.50% and 95.66%, and the low-value recovery rates were 95.34% and 88.51%. The FCBA method achieved a 100% concordance rate in detecting type 1, type 2, and type 3 vWD, as well as healthy subjects. Conclusion:A ristocetin-independent FCBA method for detecting vWF activity based on mutant GPⅠbα is successfully established.

BACKGROUND:Platelet-rich plasma is an important material for tissue repair and is widely used in orthopedics,wound repair,plastic surgery and other fields.The device for preparing platelet-rich plasma has become a hot spot in the field of patent applications.OBJECTIVE:To analyze the trend of patent applications for platelet-rich plasma separation devices at home and abroad.METHODS:Using the patent retrieval system of China National Intellectual Property Administration,Patsnap and Himpat patent databases,we searched the patent literature of platelet-rich plasma separation devices,and analyzed the patent application trend of platelet-rich plasma separation devices from the aspects of patent application volume,licensing rate,technical theme,typical applicant patent layout,etc.The patent retrieval system of China National Intellectual Property Administration was developed by the China National Intellectual Property Administration and includes patent data from 103 countries,regions and organizations.The Patsnap patent database was developed by PatSnap,Singapore and has 120 million patent data from more than 100 countries,providing multi-language translation and multi-language search.The Himpat patent database was developed by Himpat,China and includes more than 160 million patents from 123 countries/organizations/regions around the world since 1800,and is translated into high-fidelity Chinese and English versions,and is updated twice a week.RESULTS AND CONCLUSION:The patent application for platelet-rich plasma separation devices in China started about 10 years later than abroad,and the number of patent applications in China has sharply increased since 2017.The trend of patent applications abroad is relatively stable.Most domestic and foreign patent applications are in the stage of authorization and maintenance,with enterprises being the main applicants.The classification of platelet-rich plasma devices on the market is very confusing.In this study,they are divided into nine subcategories based on device structure.Chinese patent applications mainly focus on separation cylinder devices,while foreign applications focus on automated separation devices.China should be market-oriented and technology oriented,develop innovative platelet-rich plasma devices,lay out global patent layouts,and promote the development of China's medical device industry.

BACKGROUND:Platelet-rich plasma is an important material for tissue repair and is widely used in orthopedics,wound repair,plastic surgery and other fields.The device for preparing platelet-rich plasma has become a hot spot in the field of patent applications.OBJECTIVE:To analyze the trend of patent applications for platelet-rich plasma separation devices at home and abroad.METHODS:Using the patent retrieval system of China National Intellectual Property Administration,Patsnap and Himpat patent databases,we searched the patent literature of platelet-rich plasma separation devices,and analyzed the patent application trend of platelet-rich plasma separation devices from the aspects of patent application volume,licensing rate,technical theme,typical applicant patent layout,etc.The patent retrieval system of China National Intellectual Property Administration was developed by the China National Intellectual Property Administration and includes patent data from 103 countries,regions and organizations.The Patsnap patent database was developed by PatSnap,Singapore and has 120 million patent data from more than 100 countries,providing multi-language translation and multi-language search.The Himpat patent database was developed by Himpat,China and includes more than 160 million patents from 123 countries/organizations/regions around the world since 1800,and is translated into high-fidelity Chinese and English versions,and is updated twice a week.RESULTS AND CONCLUSION:The patent application for platelet-rich plasma separation devices in China started about 10 years later than abroad,and the number of patent applications in China has sharply increased since 2017.The trend of patent applications abroad is relatively stable.Most domestic and foreign patent applications are in the stage of authorization and maintenance,with enterprises being the main applicants.The classification of platelet-rich plasma devices on the market is very confusing.In this study,they are divided into nine subcategories based on device structure.Chinese patent applications mainly focus on separation cylinder devices,while foreign applications focus on automated separation devices.China should be market-oriented and technology oriented,develop innovative platelet-rich plasma devices,lay out global patent layouts,and promote the development of China's medical device industry.

Objective:To evaluate the impact of donor characteristics on the prognosis of myelodysplastic syndrome (MDS) patients undergoing haplo-identical transplantation (HIDT) .Methods:A retrospective analysis of 203 MDS patients who received HIDT was conducted to evaluate how donor factors influenced transplant outcomes.Results:In MDS patients undergoing haploidentical transplantation, donors over 50 years were associated with higher EBV reactivation (2-year cumulative incidence 42.9% vs 22.0% for <50 years old; P=0.010). Female donors were linked to increased severe chronic GVHD compared with male donors (2-year incidence 11.9% vs 4.0% ; P=0.017). Additionally, 2-year overall survival (OS) was slightly lower with female donors than male donors (56.6% vs 69.7% ), but the difference was not statistically significant ( P=0.073). Donor-recipient blood type did not affect post-transplant OS or cumulative relapse rates. Donor-recipient kinship analysis revealed that child donors, compared to haploidentical sibling or parent donors, had lower rates of grade Ⅱ–Ⅳ acute GVHD (27.2% vs 45.7% vs 53.5%, P=0.007) and 2-year EBV reactivation (13.9% vs 29.3% vs 38.9%, P=0.001). For donors under 20 years, donor gender did not significantly affect 2-year OS ( P=0.913), relapse-free survival ( P=0.716), or 100-day incidence of grade Ⅱ–Ⅳ acute GVHD ( P=0.359) . Conclusion:For MDS patients undergoing HIDT, donors over 50 should be avoided. Male and child donors are preferred, while donor gender does not significantly affect outcomes if the donor is under 20 years old.

Objective:To establish a flow cytometric bead assay (FCBA) for detecting von Willebrand factor (vWF) activity based on mutant GPⅠbα and to perform its clinical validation.Methods:Recombinant GPⅠbα protein with M239V, D235Y, and C65A point mutations was constructed and bound to flow cytometric beads. vWF in samples bound to the recombinant protein, and FITC-labeled rabbit anti-human vWF polyclonal antibody was added for detection and analysis using flow cytometry. A total of 32 patients with von Willebrand disease (10 males and 22 females, aged 26.81±6.96 years) admitted to the Department of Hematology at the First Affiliated Hospital of Soochow University from January 1, 2022, to November 30, 2023, were enrolled. Additionally, 51 healthy controls (17 males and 34 females, aged 31.56±8.98 years) were included. Plasma from 20 healthy controls was pooled in equal proportions to create standard plasma. Fourteen dilutions were prepared, ranging from the original plasma to a 1∶8, 192 dilution, and a curve was plotted according to the FCBA protocol to determine the optimal dilution. Plasma from one healthy control was aliquoted and frozen, and FCBA was performed on days 1, 5, 15, 30, 45, and 60 after bead coating to evaluate stability. The same healthy control plasma sample was tested 20 times using both FCBA and vWF enzyme-linked immunesorbent assay (ELISA) to calculate within-batch precision. The same sample was tested daily for 10 consecutive days using both FCBA and vWF-ELISA to calculate between-batch precision. All samples were tested using FCBA, and the same samples were also tested using vWF-ELISA. The χ2 test was used to compare the positive rates, negative rates, and accuracy of the two methods. One sample was used as the base sample, and another sample with a known activity of 100% was diluted to high, medium, and low activities and added to the base sample. Recovery rates were calculated using both FCBA and vWF-ELISA. Re-collected 11 cases of vWD patient samples and 10 cases of healthy subjects, and detected vWF activity using the FCBA method to validate the concordance of FCBA in clinical applications. Results:The optimal dilution for standard plasma was determined to be 1∶64. The coated beads remained stable for detection up to 60 days, with correlation coefficients of the standard curve on days 1, 5, 15, 30, 45, and 60 being 0.98, 0.98, 0.97, 0.95, 0.95, and 0.95, respectively. The within-batch coefficients of variation for FCBA and vWF-ELISA were 5.35% and 6.08%, respectively, while the between-batch coefficients of variation were 7.02% and 7.98%, respectively. The correlation coefficient between FCBA and vWF-ELISA was R2=0.798 ( P0.01). The positive rates were 90.63% and 84.38% ( χ2=0.571, P0.05), the negative rates were 92.16% and 94.12% ( χ2=0.153, P0.05), and the accuracy rates were 91.57% and 90.36% ( χ2=0.073, P0.05) for FCBA and vWF-ELISA, respectively. In the recovery experiment, the high-value recovery rates for FCBA and ELISA were 102.11% and 99.32%, respectively, the medium-value recovery rates were 98.50% and 95.66%, and the low-value recovery rates were 95.34% and 88.51%. The FCBA method achieved a 100% concordance rate in detecting type 1, type 2, and type 3 vWD, as well as healthy subjects. Conclusion:A ristocetin-independent FCBA method for detecting vWF activity based on mutant GPⅠbα is successfully established.

Platelet-derived growth factor receptor beta (PDGFRB) rearrangements play an important role in the pathogenesis of eosinophilia-associated myeloid/lymphoid neoplasms. Up to now, more than 70 PDGFRB fusions have been identified. Here, a novel PDGFRB fusion gene CSNK2A1-PDGFRB has been identified in myeloproliferative neoplasm (MPN) with eosinophilia by RNA-sequencing, which has been verified by reverse transcription polymerase chain reaction and Sanger sequencing. The new PDGFRB fusion partner gene CSNK2A1 encoded one of the two catalytic subunit of casein kinase II (CK2). To our knowledge, this is the first report on the involvement of CSNK2A1 in fusion genes, especially fusion with another kinase PDGFRB in MPN. In addition, the CSNK2A1-PDGFRB fusion retained the entire kinase domain of PDGFRB and response to imatinib at low concentration. The patient with CSNK2A1-PDGFRB was sensitive to imatinib treatment and acquired sustained complete remission.

Platelet-derived growth factor receptor beta (PDGFRB) rearrangements play an important role in the pathogenesis of eosinophilia-associated myeloid/lymphoid neoplasms. Up to now, more than 70 PDGFRB fusions have been identified. Here, a novel PDGFRB fusion gene CSNK2A1-PDGFRB has been identified in myeloproliferative neoplasm (MPN) with eosinophilia by RNA-sequencing, which has been verified by reverse transcription polymerase chain reaction and Sanger sequencing. The new PDGFRB fusion partner gene CSNK2A1 encoded one of the two catalytic subunit of casein kinase II (CK2). To our knowledge, this is the first report on the involvement of CSNK2A1 in fusion genes, especially fusion with another kinase PDGFRB in MPN. In addition, the CSNK2A1-PDGFRB fusion retained the entire kinase domain of PDGFRB and response to imatinib at low concentration. The patient with CSNK2A1-PDGFRB was sensitive to imatinib treatment and acquired sustained complete remission.

Objective: To assess the significance of DDAVP use in the diagnosis and treatment of VWD. Methods: An analysis of 15 VWD cases who referred to Hematology Division of First affiliated Hospital of Soochow University and treated with DDAVP from March 2016 to August 2018 was conducted. Efficacy and treatment response of DDAVP were monitored by observations of changes in factor Ⅷ procoagulant (FⅧ∶C) and von Willebrand Factor (VWF) related indicators before and 2 h after DDAVP injection. Results: Of 15 cases with VWD, 7 males and 8 females with a median age of 23 (6-46) years, 7 of 9 type I VWD patients achieved complete response (CR) , 1 type 2A VWD case CR, 5 type 3 VWD ones no response (NR) . The VWF multimer analysis in 5 patients combined with other plasma VWF values were in accordance with the known diagnosis. Conclusions DDAVP was effective in most type 1 patients, and ineffective in some type 2 and almost all type 3 cases. It was helpful for diagnosis and subsequent treatment planning.

Objective: To explore the correlation and consistency between thromboelastography (TEG) and traditional coagulation tests (CCTs) in ischemic cerebral vascular disease (ICVD). Methods: Totally 108 ICVD patients admitted to Nanyang Central Hospital from May 1 to October 31 2018 were enrolled. Patients’ TEG parameters (R value, K value, Angle value, MA value, CI value and G value) and CCTs parameters (PT, APTT, TT, and FIB) were collected and analyzed retrospectively. The Spearman correlation coefficient was used to explore the correlation between TEG and CCTs parameters, and Kappa (κ) to explore the consistency in determining the coagulation status of the patients. The ROC curve was used to analyze the predictive value of TEG parameters for abnormal results of CCTs, and the results of TEG and CCTs were comprehensively analyzed to evaluate the ability to predict the coagulation status of patients. Results: (1) PLT was positively correlated with MA value and G value; PT and APTT were positively correlated with K value; TT was positively correlated with R value and K value; FIB was positively correlated with Angle value, MA value and G value. TT was negatively correlated with Angle value and CI value; FIB was negatively correlated with K value. (2) PT and MA values, PT and G values, FIB and MA values, FIB and G values were accordant in valuing the hypoxic state of ICVD patients. (3) PLT and Angle values, PLT and MA values, PLT and CI values, PLT and G values were accordant in assessing hypercoagulable status of ICVD patients; FIB and Angle values, FIB and MA values, FIB and CI value, and FIB and G value were consistent in evaluating the hypercoagulable state of ICVD patients. (4) For detecting TT>20 s, the AUC of K value and Angle value were 0.648, 0.651, respectively; For detecting FIB>4 g/L, the AUC of Angle value and MA value were 0.717 and 0.747, respectively; For detecting PLT>300×10⁹/L, the AUC of MA value was 0.808 (all P<0.05). Conclusions There is weak correlation and consistency between TEG and CCTs parameters in ICVD patients. The TEG parameters have good predictive value in evaluating the abnormal results of CCTs, but cannot replace the CCTs. Combination of these two methods can better reflect the coagulation status of patients, so as to afford assistance.