International journal of laboratory hematology最新文献

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VEXAS Syndrome Associated With a Non-Canonical UBA1 p.Ala478Ser Variant: Diagnostic and Clinical Implications. 与非典型UBA1 p.Ala478Ser变异相关的VEXAS综合征:诊断和临床意义
International journal of laboratory hematology Pub Date : 2026-09-30 DOI: 10.1111/ijlh.70254
Ilaria Scarpelli, Mario Uhr, Leda Leoncini, Ida Martinelli
{"title":"VEXAS Syndrome Associated With a Non-Canonical UBA1 p.Ala478Ser Variant: Diagnostic and Clinical Implications.","authors":"Ilaria Scarpelli, Mario Uhr, Leda Leoncini, Ida Martinelli","doi":"10.1111/ijlh.70254","DOIUrl":"https://doi.org/10.1111/ijlh.70254","url":null,"abstract":"","PeriodicalId":94050,"journal":{"name":"International journal of laboratory hematology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149120892","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Comparison of the HemoScreen Point-Of-Care Haematology Analyser With Sysmex XN, Beckman Coulter DxH 560 and HORIBA Yumizen H550 in Patients With Abnormal Blood Counts. haemscreen即时血液学分析仪与Sysmex XN、Beckman Coulter DxH 560和HORIBA Yumizen H550在血液计数异常患者中的比较
International journal of laboratory hematology Pub Date : 2026-09-25 DOI: 10.1111/ijlh.70253
Katarína Hradská, Martin Pulcer, Michaela Skořupová, Tereza Popková, Michal Kaščák, Jana Mihályová, Ivo Demel, Barbora Dluhošová, Kateřina Benková, Juraj Ďuraš, Soňa Kolářová, Veronika Hanzelková, Simona Pulcerová, Roman Hájek
{"title":"Comparison of the HemoScreen Point-Of-Care Haematology Analyser With Sysmex XN, Beckman Coulter DxH 560 and HORIBA Yumizen H550 in Patients With Abnormal Blood Counts.","authors":"Katarína Hradská, Martin Pulcer, Michaela Skořupová, Tereza Popková, Michal Kaščák, Jana Mihályová, Ivo Demel, Barbora Dluhošová, Kateřina Benková, Juraj Ďuraš, Soňa Kolářová, Veronika Hanzelková, Simona Pulcerová, Roman Hájek","doi":"10.1111/ijlh.70253","DOIUrl":"https://doi.org/10.1111/ijlh.70253","url":null,"abstract":"<p><strong>Introduction: </strong>HemoScreen is a portable point-of-care testing (POCT) haematology analyser capable of measuring 20 complete blood count (CBC) parameters, including a 5-part leukocyte differential, from venous and capillary blood. This study compared selected CBC and white blood cell (WBC) differential parameters obtained using HemoScreen with those from three conventional haematology analysers: Sysmex XN, Beckman Coulter DxH 560, and HORIBA Yumizen H550 in abnormal samples.</p><p><strong>Methods: </strong>Venous and capillary blood was collected from 137 patients with haematological disorders and healthy donors. We evaluated WBC, absolute neutrophil count (ANC), haemoglobin (HGB), and platelets (PLT). Statistical analysis included Passing-Bablok regression analysis, Cusum test, Wilcoxon signed-rank test, and Hodges-Lehmann median difference analysis.</p><p><strong>Results: </strong>Comparison of venous and capillary blood measurements using HemoScreen demonstrated no significant deviation from linearity for any evaluated parameter according to the Cusum test (p = 0.14-0.98). Although statistically significant differences were observed for ANC (p = 0.0201) and PLT (p = 0.0026), the median differences remained small and clinically irrelevant. Comparison of venous blood measurements between HemoScreen and conventional analysers demonstrated strong analytical agreement without significant deviation from linearity. Statistically significant differences were observed in several comparisons, particularly for PLT and HGB measurements; however, the approximate relative bias remained within external quality assessment limits in most comparisons, with greater variability for PLT in the ≤ 50 × 10<sup>9</sup>/L subgroup.</p><p><strong>Conclusions: </strong>HemoScreen demonstrated good analytical agreement with all evaluated haematology analysers and may represent a reliable complementary POCT tool for routine haematological monitoring, including patients with abnormal blood counts.</p>","PeriodicalId":94050,"journal":{"name":"International journal of laboratory hematology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149066885","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Differentiating β-Thalassaemia From Iron Deficiency: Performance and Cut-Off Dependency of More Than 40 Red Cell Discrimination Indices in a Large German Cohort. 区分β-地中海贫血与缺铁:在一个大型德国队列中超过40个红细胞鉴别指标的表现和切断依赖性
International journal of laboratory hematology Pub Date : 2026-09-23 DOI: 10.1111/ijlh.70252
Rafid Al-Nabhan, Dani Hakimeh, Lena Oevermann
{"title":"Differentiating β-Thalassaemia From Iron Deficiency: Performance and Cut-Off Dependency of More Than 40 Red Cell Discrimination Indices in a Large German Cohort.","authors":"Rafid Al-Nabhan, Dani Hakimeh, Lena Oevermann","doi":"10.1111/ijlh.70252","DOIUrl":"https://doi.org/10.1111/ijlh.70252","url":null,"abstract":"<p><strong>Background: </strong>Since the 1970s, more than 40 red blood cell-based formulas have been proposed to differentiate β-thalassaemia trait (BTT) from iron deficiency (ID) and iron deficiency anaemia (IDA), the main causes of microcytic anaemia. In Germany, where haemoglobinopathies are rare in the autochthonous population but increasingly encountered due to migration, rapid, low-cost screening tools are of growing importance. This study benchmarked more than 40 published discrimination indices (1973-2024) in a large laboratory dataset and explored new formulas.</p><p><strong>Methods: </strong>In a retrospective analysis, 1316 of 5715 screened records met predefined criteria and were stratified into a BTT group (n = 953) and an ID/IDA group (n = 363). Diagnostic performance was assessed using sensitivity, specificity, Youden's index and ROC analysis.</p><p><strong>Results: </strong>No established index reliably separated BTT from ID/IDA, and performance was strongly cut-off-dependent: the best indices reached AUCs of approximately 0.80 with their published cut-offs (e.g., Wongprachum, 0.803) but up to 0.879 on cut-off-independent ROC analysis (Jayabose), indicating that published cut-offs are not directly transferable between populations. In the independent test dataset, three newly derived formulas performed comparably to the best published indices (AUCs 0.916-0.923 vs. up to 0.915); rather than outperforming existing models, their advantage lies in offering selectable cut-offs for sensitivity-, specificity- or balance-oriented use.</p><p><strong>Conclusions: </strong>Local validation of discrimination indices and cut-offs is essential before clinical use. Simple CBC-based formulas may serve as low-cost preselection tools to identify individuals requiring further evaluation. As the new formulas were validated internally only, external validation is required before clinical application.</p>","PeriodicalId":94050,"journal":{"name":"International journal of laboratory hematology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-09-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149027195","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Adding-On Wisely: Hematopathology Reflective Testing in Canada. 明智地补充:血液病反射测试在加拿大。
International journal of laboratory hematology Pub Date : 2026-09-22 DOI: 10.1111/ijlh.70248
Eric McEvoy, Eileen R McBride
{"title":"Adding-On Wisely: Hematopathology Reflective Testing in Canada.","authors":"Eric McEvoy, Eileen R McBride","doi":"10.1111/ijlh.70248","DOIUrl":"https://doi.org/10.1111/ijlh.70248","url":null,"abstract":"<p><strong>Introduction: </strong>Reflective testing describes laboratory clinicians' discretionary addition of testing on existing samples, after initial result review. This practice has proven valuable in clinical biochemistry but faces controversy regarding consent and costs. No prior research exists regarding reflective testing in Canadian hematopathology. We surveyed hematopathologists across Canada to characterize current practices and perspectives.</p><p><strong>Methods: </strong>A REDCap survey was distributed to 89 licensed hematopathologists across all provinces (excluding Quebec), capturing demographics, institutional guidelines, and reflective testing practices, as well as ethical and medicolegal perspectives. Descriptive statistical analysis was performed on all responses.</p><p><strong>Results: </strong>Thirty-three eligible survey responses were received. Reflective testing was ordered at least once weekly by 63% of hematopathologists, yet only 30% had institutional guidance. Ethical perspectives varied: 27% felt that consenting to initial testing implies consent for reflective testing, while 61% indicated this depends on the type of test added. Routine prior notification of the initial ordering clinician (IOC) and/or patient was reported by only 24%. Overall, 79% supported the use of reflective testing, citing improved diagnostic timeliness, reduced phlebotomy, and patient management. None opposed the use of reflective testing, although 21% had encountered a complaint or legal concern related to the practice.</p><p><strong>Conclusion: </strong>Reflective testing is widely used and supported among Canadian hematopathologists as a value-added practice leveraging clinical expertise to improve patient care. Implementation varies, with diverse institutional guidance and perspectives on implied consent, communication, and medicolegal responsibility. Our findings identify an opportunity for national consensus building to support transparent, ethically grounded, and patient-centered reflective testing practices in hematopathology.</p>","PeriodicalId":94050,"journal":{"name":"International journal of laboratory hematology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-09-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149019019","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Verification of Patient Result Comparability Among Nine Hematology Analyzers Using the CLSI EP31-A-IR Guideline: A Four-Year Experience. 使用CLSI EP31-A-IR指南验证九种血液分析仪患者结果的可比性:四年经验
International journal of laboratory hematology Pub Date : 2026-09-15 DOI: 10.1111/ijlh.70243
Xavier Tejedor-Ganduxé, Fernando Marqués-García, Cristina Martínez-Bravo, Isabel Aparicio-Calvente, Laura Jiménez-Añon, Alba Leis-Sestayo, Jennifer Rodríguez-Domínguez, Alicia Martínez-Iribarren, Cristian Morales-Indiano
{"title":"Verification of Patient Result Comparability Among Nine Hematology Analyzers Using the CLSI EP31-A-IR Guideline: A Four-Year Experience.","authors":"Xavier Tejedor-Ganduxé, Fernando Marqués-García, Cristina Martínez-Bravo, Isabel Aparicio-Calvente, Laura Jiménez-Añon, Alba Leis-Sestayo, Jennifer Rodríguez-Domínguez, Alicia Martínez-Iribarren, Cristian Morales-Indiano","doi":"10.1111/ijlh.70243","DOIUrl":"https://doi.org/10.1111/ijlh.70243","url":null,"abstract":"<p><strong>Introduction: </strong>Clinical laboratories operating multiple analyzers with random sample allocation must ensure result comparability across instruments to support consistent clinical interpretation and patient safety.</p><p><strong>Objectives: </strong>To evaluate the applicability of the CLSI EP31-A-IR guideline for periodic verification of patient result comparability across multiple hematology analyzers, and to assess the longitudinal stability of this model over a four-year period.</p><p><strong>Methods: </strong>Comparability of nine DxH-900 hematology analyzers (Beckman Coulter) was evaluated for 17 parameters, including complete blood count variables, leukocyte differentials, monocyte distribution width (MDW), and reticulocytes. Following the EP31-A-IR framework, approximate target concentrations were estimated from internal quality control data at three concentration levels to guide the selection of native patient samples covering clinically relevant ranges, including samples from oncohematological and sepsis-suspected patients. Acceptance criteria (AC) based on biological variation (BV) were established for each parameter and concentration level. The maximum allowable difference (MAD), as well as the number of runs and replicates, were calculated accordingly. Results were considered comparable when the maximum observed difference (MOD) was less than or equal to the MAD.</p><p><strong>Results: </strong>For all 17 parameters evaluated, including reticulocytes assessed on a subset of four analyzers reflecting routine workflow, the MOD between instruments remained within predefined acceptance limits at all concentration levels. Longitudinal monitoring over a four-year period (2022-2025) demonstrated sustained inter-analyzer agreement, with no evidence of progressive analytical drift.</p><p><strong>Conclusions: </strong>The EP31-A-IR guideline proved to be a flexible and effective framework for periodic verification of patient result comparability in high-volume hematology laboratories. This study represents one of the first implementations of this guideline in the field of hematology, supporting reliable clinical interpretation across analytical platforms and fulfilling ISO 15189 accreditation requirements.</p>","PeriodicalId":94050,"journal":{"name":"International journal of laboratory hematology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-09-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148972725","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Characterizing Red Blood Cell Rheological Parameters Using Automated Syllectometry: Conventional and Exploratory Multivariate Analyses. 表征红细胞流变参数使用自动分子计量:常规和探索性的多变量分析。
International journal of laboratory hematology Pub Date : 2026-09-15 DOI: 10.1111/ijlh.70251
Baran Dastaran, Yousra Tera, Maica Jhiezl Yunon, Maha Othman
{"title":"Characterizing Red Blood Cell Rheological Parameters Using Automated Syllectometry: Conventional and Exploratory Multivariate Analyses.","authors":"Baran Dastaran, Yousra Tera, Maica Jhiezl Yunon, Maha Othman","doi":"10.1111/ijlh.70251","DOIUrl":"https://doi.org/10.1111/ijlh.70251","url":null,"abstract":"<p><strong>Introduction: </strong>Red blood cell (RBC) biomechanics are key determinants of blood rheology, microcirculatory flow, and tissue oxygen delivery, yet remain underutilized in clinical practice because of methodological variability and limited standardization. This study aimed to define preliminary reference distributions for RBC rheological parameters using the automated MIZAR syllectometry platform and to characterize inter-parameter relationships using exploratory multivariate analyses.</p><p><strong>Methods: </strong>Whole blood samples from 70 healthy adults were analyzed to generate 13 MIZAR parameters reflecting RBC deformability, aggregation, and elasticity. Conventional statistical methods were used to define reference distributions and assess demographic influences. Principal component analysis (PCA), hierarchical clustering, and correlation analysis were applied to explore covariance structure and statistical interrelationships within the dataset.</p><p><strong>Results: </strong>Within-run precision was high for several MIZAR parameters, particularly at 800 nm, with mean coefficients of variation < 2% for AI, Int(a), AMP, T1/2, OTF, OT, and ED. Most parameters demonstrated minimal age-related variation. Several parameters differed by sex, particularly those related to aggregation amplitude and optical measurements. PCA showed that the first two components explained 60.9% of total variance, while hierarchical clustering identified five exploratory parameter groupings broadly consistent with their measured rheological characteristics. Correlation analysis further characterized inter-parameter associations.</p><p><strong>Conclusion: </strong>These findings provide the first preliminary reference dataset for MIZAR-derived RBC parameters and identify patterns of covariance and interrelationships within a healthy cohort. Integrating conventional and exploratory multivariate analyses provides a framework for characterizing interrelated hemorheological measurements. Validation in independent and clinically diverse populations is required to determine the reproducibility and clinical utility of these findings.</p>","PeriodicalId":94050,"journal":{"name":"International journal of laboratory hematology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-09-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148972718","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Is It Time to Raise the Threshold for Critically Low Fibrinogen? Insights From a Retrospective, Consecutive-Case Cohort Study of Low Fibrinogen due to Various Causes. 是时候提高纤维蛋白原临界值了吗?来自各种原因导致的低纤维蛋白原回顾性、连续病例队列研究的见解。
International journal of laboratory hematology Pub Date : 2026-09-11 DOI: 10.1111/ijlh.70246
Natalie Mathews, Subia Tasneem, Mana Al Dawsari, Keona Pang, Yasmeen Abulkhair, Guillame Paré, Siraj Mithoowani, Catherine P M Hayward
{"title":"Is It Time to Raise the Threshold for Critically Low Fibrinogen? Insights From a Retrospective, Consecutive-Case Cohort Study of Low Fibrinogen due to Various Causes.","authors":"Natalie Mathews, Subia Tasneem, Mana Al Dawsari, Keona Pang, Yasmeen Abulkhair, Guillame Paré, Siraj Mithoowani, Catherine P M Hayward","doi":"10.1111/ijlh.70246","DOIUrl":"https://doi.org/10.1111/ijlh.70246","url":null,"abstract":"<p><strong>Introduction: </strong>Fibrinogen deficiency is an important coagulation abnormality, with diverse causes.</p><p><strong>Methods: </strong>A consecutive-case cohort study of adult and pediatric patients with low Clauss fibrinogen at four acute care hospitals was undertaken to explore findings, causes, and predictors of outcomes, including the optimal fibrinogen critical value to predict all-cause mortality.</p><p><strong>Results: </strong>Over 11 months, 4.9% of tested patients (349/7183, after excluding n = 4 for preanalytical errors) had fibrinogen deficiency (63.6% male; 86.8% adult; 29.2% with liver disease; 14.0% with massive transfusion; 19.5% with fibrinogen < 1.0 g/L, the historical critical value; none with a diagnosed inherited deficiency). Primary causes of fibrinogen deficiency were: cardiovascular surgery (CVS); trauma; overt, not overt and potential/not scorable (no D-dimer results) disseminated intravascular coagulation (DIC); liver disease; acute bleeding; and other (including uncertain) causes. After Bonferroni correction, overt and potential/not scorable DIC were associated with significantly lower fibrinogen levels and higher mortality than most other causes were, particularly compared to trauma (p < 0.0001). Median time to death after diagnosing fibrinogen deficiency was 1 (IQR: < 1-7) day. A Clauss fibrinogen ≤ 1.3 g/L had optimal sensitivity and specificity for predicting mortality among fibrinogen-deficient patients, and multivariate analyses indicated it was a strong, independent predictor of death (adjusted odds ratio [95% confidence intervals]: 6.2 [2.9-13.6] vs. ≤ 3.6 for liver disease and DIC). Spearman correlation indicated that the nadir Clauss fibrinogen level had a negative correlation (r = -0.34, p < 0.0001) to the amount of fibrinogen replacement received.</p><p><strong>Conclusions: </strong>Low Clauss fibrinogen was commonly acquired and levels ≤ 1.3 g/L were optimal for predicting all-cause mortality.</p>","PeriodicalId":94050,"journal":{"name":"International journal of laboratory hematology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148957648","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The Concept of "Reference" for Individuals and Populations: Why Personalized Reference Intervals Are Frequently Misunderstood. 个人和群体的“参考”概念:为什么个性化参考区间经常被误解。
International journal of laboratory hematology Pub Date : 2026-09-09 DOI: 10.1111/ijlh.70250
Abdurrahman Coşkun, Özlem Demirelce
{"title":"The Concept of \"Reference\" for Individuals and Populations: Why Personalized Reference Intervals Are Frequently Misunderstood.","authors":"Abdurrahman Coşkun, Özlem Demirelce","doi":"10.1111/ijlh.70250","DOIUrl":"https://doi.org/10.1111/ijlh.70250","url":null,"abstract":"","PeriodicalId":94050,"journal":{"name":"International journal of laboratory hematology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-09-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148914863","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Functional Assessment of Platelet-Derived Phospholipid Activity in Pooled Normal Plasmas by Low-Phospholipid Thrombin Generation Assay and Its Impact on Lupus Anticoagulant Mixing Tests. 用低磷脂凝血酶生成法评价正常血浆血小板源性磷脂活性及其对狼疮抗凝血混合试验的影响
International journal of laboratory hematology Pub Date : 2026-09-07 DOI: 10.1111/ijlh.70247
Osamu Kumano, Masato Matsuda, Mino Sakata, Osamu Maruyama
{"title":"Functional Assessment of Platelet-Derived Phospholipid Activity in Pooled Normal Plasmas by Low-Phospholipid Thrombin Generation Assay and Its Impact on Lupus Anticoagulant Mixing Tests.","authors":"Osamu Kumano, Masato Matsuda, Mino Sakata, Osamu Maruyama","doi":"10.1111/ijlh.70247","DOIUrl":"https://doi.org/10.1111/ijlh.70247","url":null,"abstract":"<p><strong>Background: </strong>Pooled normal plasma (PNP) used for lupus anticoagulant (LA) mixing tests may carry platelet-derived phospholipid (PL) activity introduced during freezing/lyophilization, potentially attenuating LA-mediated prolongation of clotting times.</p><p><strong>Objectives: </strong>To determine whether thrombin generation assay (TGA) under low-PL conditions functionally captures endogenous, platelet-derived PL activity in commercial PNP and relates to LA mixing outcomes.</p><p><strong>Methods: </strong>Four commercial PNPs (CRYOcheck PNP, Coagtrol N, SHP, Ci-Trol 1) were tested by TGA at PL 4, 1, 0.1, and 0 μM with 1 pM tissue factor and 1.6 μM corn trypsin inhibitor. LA weak-positive and LA positive controls were mixed 1:1 with each PNP and assessed by five APTT reagents to compute the index of circulating anticoagulant (ICA). Platelet factor 4 (PF4) levels were measured by ELISA.</p><p><strong>Results: </strong>SHP and Ci-Trol 1 showed consistently higher TGA peak heights than CRYOcheck PNP and Coagtrol N across all PL concentrations; thrombin generation persisted at 0 μM PL, consistent with activity in the absence of added PL. The other TGA parameters exhibited the same trend. Mean ICA values were inversely correlated with TGA peak height at 4 and 1 μM PL (r ≤ -0.985), with direction preserved at 0.1 and 0 μM. PF4 levels were markedly higher in SHP and Ci-Trol 1 and paralleled higher TGA peak height and lower ICA.</p><p><strong>Conclusions: </strong>Endogenous, platelet-derived PL activity in commercial PNP is a key determinant of the LA mixing test performance. TGA under low-PL conditions, complemented by PF4 measurement, offers a practical approach to qualify PNP lots and reduce the under recognition of LA.</p>","PeriodicalId":94050,"journal":{"name":"International journal of laboratory hematology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-09-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148907928","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Utilizing Inhibitor Screening Assay Results to Predict Bethesda Titers in Patients With Hemophilia A: Cost-Effective Strategy in Resource-Constrained Setting. 利用抑制剂筛选试验结果预测血友病A患者的Bethesda滴度:资源受限环境下的成本效益策略。
International journal of laboratory hematology Pub Date : 2026-09-06 DOI: 10.1111/ijlh.70233
Dinesh Chandra, Manish Kumar Singh, Ruchi Gupta, Khaliqur Rahman, Prakhar Gupta, Anup Kumar, Jayanta Kumar Biswas, Yadav Sanjeev, Rajesh Kashyap
{"title":"Utilizing Inhibitor Screening Assay Results to Predict Bethesda Titers in Patients With Hemophilia A: Cost-Effective Strategy in Resource-Constrained Setting.","authors":"Dinesh Chandra, Manish Kumar Singh, Ruchi Gupta, Khaliqur Rahman, Prakhar Gupta, Anup Kumar, Jayanta Kumar Biswas, Yadav Sanjeev, Rajesh Kashyap","doi":"10.1111/ijlh.70233","DOIUrl":"10.1111/ijlh.70233","url":null,"abstract":"<p><strong>Background: </strong>The classical or modified Nijmegen-Bethesda assay measures FVIII inhibitor titers and requires testing residual FVIII levels at multiple serial dilutions, making it prohibitively expensive in resource-constrained settings. The study aimed to develop a cost-effective strategy to predict inhibitor titers (Bethesda units [BU]) in patients with hemophilia A (HA) using the inhibitor screening assay.</p><p><strong>Methods: </strong>Inhibitor screening and Bethesda assay were performed on samples of suspected HA with inhibitors. The laboratory data were analyzed to develop a model to predict inhibitor titers from inhibitor screening (normalized ratio of APTT values of the fresh mix [FM] or incubated mix [IM]). The model was then prospectively validated on new inhibitor-positive samples.</p><p><strong>Results: </strong>One hundred and four samples from 83 patients with HA and inhibitors were analyzed. A linear regression analysis was performed, and the normalized APTT ratio of FM (R<sup>2</sup> = 0.815) proved to be highly predictive of inhibitor titers (BU) compared with IM (R<sup>2</sup> = 0.536). A predictive model was developed based on the normalized APTT ratio of FM values. This predictive model was then validated on a new cohort of 27 inhibitor-positive samples, where the predicted inhibitor titers strongly correlated with the observed titers (R<sup>2</sup> = 0.843).</p><p><strong>Conclusion: </strong>The FM of the screening assay serves as a potential indicator of the expected inhibitor titers (BU) and guides the selection of dilutions likely to yield near-50% residual FVIII activity for estimating the Bethesda titers. This approach substantially reduces assay costs, particularly in cases with > 2 BU. Additional prospective studies are needed to validate these results.</p>","PeriodicalId":94050,"journal":{"name":"International journal of laboratory hematology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-09-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148901495","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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