Pub Date : 2025-02-01Epub Date: 2024-07-01DOI: 10.1111/ijlh.14336
Qiang Meng, Yang Fu
{"title":"Leukocytes engulfing platelets: New evidence of thrombocytopenia in autoimmune necrotic myositis from morphological observation.","authors":"Qiang Meng, Yang Fu","doi":"10.1111/ijlh.14336","DOIUrl":"10.1111/ijlh.14336","url":null,"abstract":"","PeriodicalId":94050,"journal":{"name":"International journal of laboratory hematology","volume":" ","pages":"5-7"},"PeriodicalIF":0.0,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141478254","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}
Pub Date : 2025-02-01Epub Date: 2024-10-04DOI: 10.1111/ijlh.14375
Shalini Goel, Aseem Kumar Tiwari, Pawan Kumar Gahlot, Manish Kumar Singh, Renu Saxena, Vaibhav Jadhav, Monisha Sethi, Tan Swee Jin
Introduction: Complete blood count is the most common, basic test requisitioned in hematology. The normal reference ranges of hematological parameters are required owing to variable socioeconomic, environmental, and genetic factors in populations. The current study determines the reference ranges of the healthy Indian donor population of a high socioeconomic group.
Methods: The study was conducted in the Department of Transfusion Medicine at a tertiary care hospital in India and included 4098 individuals, aged 18-65 years coming for voluntary blood donation from July 2021 to October 2022. Blood samples were collected in K2EDTA, analyzed on the Sysmex XN-1500 hematology analyzer, and using statistical tools, the normal reference ranges were calculated.
Results: The reference ranges for hemoglobin (HB) (137-185 g/L), WBC (5.1-11.7 × 109/L), platelet count (115.6-370.0 × 109/L) were noted. [Correction added on 22 10 2024, after first online publication: The WBC value has been corrected in this version.] No statistically significant changes were observed in different age groups. There were gender-wise differences noted in nearly all parameters. The HB and hematocrit (HCT) range was slightly higher in other Indian and other Asian populations with comparable values with the Chinese, Korean populations, and Western populations; RBC parameters were overall comparable with minor differences; the WBC count was higher than the other Indian and Asian populations particularly the upper limit of lymphocyte and monocyte; and the range of platelet counts had a comparable upper limit with all populations and had the lowest lower value in males in our study, which was comparable to only the Chinese population.
Conclusions: It is concluded that reference ranges of common parameters were calculated with minor changes noted in all hematological parameters on comparing with other Indian, Asian population, and Western data.
{"title":"Biological Reference Ranges for Healthy Indian Blood Donors: Experience of a Tertiary Care Center Vis-à-Vis International Literature.","authors":"Shalini Goel, Aseem Kumar Tiwari, Pawan Kumar Gahlot, Manish Kumar Singh, Renu Saxena, Vaibhav Jadhav, Monisha Sethi, Tan Swee Jin","doi":"10.1111/ijlh.14375","DOIUrl":"10.1111/ijlh.14375","url":null,"abstract":"<p><strong>Introduction: </strong>Complete blood count is the most common, basic test requisitioned in hematology. The normal reference ranges of hematological parameters are required owing to variable socioeconomic, environmental, and genetic factors in populations. The current study determines the reference ranges of the healthy Indian donor population of a high socioeconomic group.</p><p><strong>Methods: </strong>The study was conducted in the Department of Transfusion Medicine at a tertiary care hospital in India and included 4098 individuals, aged 18-65 years coming for voluntary blood donation from July 2021 to October 2022. Blood samples were collected in K2EDTA, analyzed on the Sysmex XN-1500 hematology analyzer, and using statistical tools, the normal reference ranges were calculated.</p><p><strong>Results: </strong>The reference ranges for hemoglobin (HB) (137-185 g/L), WBC (5.1-11.7 × 10<sup>9</sup>/L), platelet count (115.6-370.0 × 10<sup>9</sup>/L) were noted. [Correction added on 22 10 2024, after first online publication: The WBC value has been corrected in this version.] No statistically significant changes were observed in different age groups. There were gender-wise differences noted in nearly all parameters. The HB and hematocrit (HCT) range was slightly higher in other Indian and other Asian populations with comparable values with the Chinese, Korean populations, and Western populations; RBC parameters were overall comparable with minor differences; the WBC count was higher than the other Indian and Asian populations particularly the upper limit of lymphocyte and monocyte; and the range of platelet counts had a comparable upper limit with all populations and had the lowest lower value in males in our study, which was comparable to only the Chinese population.</p><p><strong>Conclusions: </strong>It is concluded that reference ranges of common parameters were calculated with minor changes noted in all hematological parameters on comparing with other Indian, Asian population, and Western data.</p>","PeriodicalId":94050,"journal":{"name":"International journal of laboratory hematology","volume":" ","pages":"61-67"},"PeriodicalIF":0.0,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142373930","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}
Pub Date : 2025-02-01Epub Date: 2024-10-21DOI: 10.1111/ijlh.14387
Suhyeon Woo, Bohyun Kim, Nam Hun Heo, Min-Sun Kim, Young Ahn Yoon, Young-Jin Choi
Objectives: This study aimed to determine a definition for significant platelet clumping (PC) and evaluate the performance of the Sysmex XN instrument for detecting platelet clumps.
Methods: For part 1, 372 specimens with a 'PLT_clump?' flag in XN-9000 were classified into five groups according to the average number of PCs. We compared the initial platelet count (measured by XN-9000 using impedance method) and corrected platelet count (counted optically or re-analyzed by XN-9000 using vortexed or re-collected sample) of each group. For part 2, 1000 specimens with a PC flag divided into three subgroups {group N (PC = 0), Y (PC ≥ 1), and Z (microscopic fibrin clot)} and additional two groups {group S (PC(+) specimens without any flag and with flags of other categories) and group NC (negative control)} were collected. Positive predictive value (PPV), negative predictive value (NPV), sensitivity and specificity of PC detection of XN-9000 were obtained and the platelet counts and four indices (PDW, MPV, P_LCR, and PCT) of groups NC, N, Y, Z, and S were compared to detect PC more precisely.
Results: In part 1, all groups showed significant difference between the initial and corrected platelet counts. In part 2, PPV, NPV, prevalence, sensitivity, and specificity were 41.5%, 56.5%, 43.4%, 2.18%, and 98.3%, respectively. The platelet counts and four indices showed statistical differences for detecting PCs, and especially PDW and P_LCR were significantly smaller in group Z than group N or Y.
Conclusions: We suggest the definition of significant PC by the presence of at least three platelets. In addition, utilizing platelet-related indices should be developed to improve the efficiency of the PC detection.
研究目的本研究旨在确定明显血小板聚集(PC)的定义,并评估 Sysmex XN 仪器检测血小板聚集的性能:第一部分:根据 PC 的平均数量,将 372 份在 XN-9000 中带有 "PLT_clump? "标志的标本分为五组。我们比较了每组的初始血小板计数(XN-9000 使用阻抗法测量)和校正血小板计数(XN-9000 使用涡旋或重新采集的样本进行光学计数或重新分析)。第二部分收集了 1000 份带有 PC 标志的标本,分为三个亚组{N 组(PC = 0)、Y 组(PC ≥ 1)和 Z 组(显微镜下纤维蛋白凝块)},以及另外两组{S 组(无任何标志和带有其他类别标志的 PC(+) 标本)和 NC 组(阴性对照)}。得出 XN-9000 检测 PC 的阳性预测值(PPV)、阴性预测值(NPV)、灵敏度和特异性,并比较 NC、N、Y、Z 和 S 组的血小板计数和四项指数(PDW、MPV、P_LCR 和 PCT),以更精确地检测 PC:结果:在第一部分中,所有组的初始血小板计数和校正血小板计数均有显著差异。在第二部分中,PPV、NPV、患病率、敏感性和特异性分别为 41.5%、56.5%、43.4%、2.18% 和 98.3%。血小板计数和四项指数在检测 PC 方面存在统计学差异,尤其是 Z 组的 PDW 和 P_LCR 明显小于 N 组或 Y 组:结论:我们建议以至少出现三个血小板来定义明显的 PC。结论:我们建议以至少出现三个血小板来定义明显的 PC,此外,应开发利用血小板相关指数来提高 PC 的检测效率。
{"title":"Definition of Significant Platelet Clumping: Should We Review All Samples With a Platelet Clumping Flag From Automated Hematology Analyzer?","authors":"Suhyeon Woo, Bohyun Kim, Nam Hun Heo, Min-Sun Kim, Young Ahn Yoon, Young-Jin Choi","doi":"10.1111/ijlh.14387","DOIUrl":"10.1111/ijlh.14387","url":null,"abstract":"<p><strong>Objectives: </strong>This study aimed to determine a definition for significant platelet clumping (PC) and evaluate the performance of the Sysmex XN instrument for detecting platelet clumps.</p><p><strong>Methods: </strong>For part 1, 372 specimens with a 'PLT_clump?' flag in XN-9000 were classified into five groups according to the average number of PCs. We compared the initial platelet count (measured by XN-9000 using impedance method) and corrected platelet count (counted optically or re-analyzed by XN-9000 using vortexed or re-collected sample) of each group. For part 2, 1000 specimens with a PC flag divided into three subgroups {group N (PC = 0), Y (PC ≥ 1), and Z (microscopic fibrin clot)} and additional two groups {group S (PC(+) specimens without any flag and with flags of other categories) and group NC (negative control)} were collected. Positive predictive value (PPV), negative predictive value (NPV), sensitivity and specificity of PC detection of XN-9000 were obtained and the platelet counts and four indices (PDW, MPV, P_LCR, and PCT) of groups NC, N, Y, Z, and S were compared to detect PC more precisely.</p><p><strong>Results: </strong>In part 1, all groups showed significant difference between the initial and corrected platelet counts. In part 2, PPV, NPV, prevalence, sensitivity, and specificity were 41.5%, 56.5%, 43.4%, 2.18%, and 98.3%, respectively. The platelet counts and four indices showed statistical differences for detecting PCs, and especially PDW and P_LCR were significantly smaller in group Z than group N or Y.</p><p><strong>Conclusions: </strong>We suggest the definition of significant PC by the presence of at least three platelets. In addition, utilizing platelet-related indices should be developed to improve the efficiency of the PC detection.</p>","PeriodicalId":94050,"journal":{"name":"International journal of laboratory hematology","volume":" ","pages":"79-86"},"PeriodicalIF":0.0,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11725553/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142484181","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-02-01Epub Date: 2024-09-02DOI: 10.1111/ijlh.14365
Pei-Chun Shih, Yi-Hua Wang, Shey-Ying Chen, Min Tseng, Cheng-An Hsu, Ming-Yan Yang, Hsin-Yao Wang, Jia-Arng Lee
Objectives: Immature platelet fraction (IPF) for differentiating bacteremia has been explored, whereas its prognostic correlation remains uncertain. This study aims to confirm the predictive capability of IPF for bacteremia and investigate its association with prognosis.
Methods: Patients with complete blood count (CBC) on the blood culture day (D1) and the preceding day (D0) were retrospectively recruited and categorized into bacteremia and nonbacteremia groups. Immature platelet (IP) analysis, alongside CBC, was conducted. Delta IPF, defined by the absolute values of D1 minus D0 results was calculated. The ability to distinguish bacteremia from nonbacteremia patients, and the correlation with mortality were analyzed.
Results: From February to December 2020, a total of 150 patients were enrolled, with 75 having bacteremia. The specificity for delta IPF ≥3.4% to predict bacteremia was 97.3% (95% confidence interval [CI]: 90.7-99.7). When delta IPF ≥3.4% combined with procalcitonin ≥0.5 (ng/mL), the sensitivity was 90.5% (95% CI: 69.6%-98.8%). Within the bacteremia group, delta IPF and the proportion of patients with delta IPF ≥1.5% were significantly higher in nonsurvival, while delta platelet levels did not. Furthermore, delta IPF ≥1.5% was independently associated with 30-day mortality (adjusted odds ratio: 3.88, 95% CI: 1.2%-11.4%; p = 0.020). The 30-day survival curve demonstrated a significant difference between patients with delta IPF ≥1.5% and those without (p < 0.001).
Conclusions: Delta IPF correlates with mortality in bacteremia patients. Our findings suggest IPF not only helps detect bacteremia but also predicts prognosis in the early stage.
{"title":"Delta Immature Platelet Fraction Is Associated With Mortality in Bacteremia Patients.","authors":"Pei-Chun Shih, Yi-Hua Wang, Shey-Ying Chen, Min Tseng, Cheng-An Hsu, Ming-Yan Yang, Hsin-Yao Wang, Jia-Arng Lee","doi":"10.1111/ijlh.14365","DOIUrl":"10.1111/ijlh.14365","url":null,"abstract":"<p><strong>Objectives: </strong>Immature platelet fraction (IPF) for differentiating bacteremia has been explored, whereas its prognostic correlation remains uncertain. This study aims to confirm the predictive capability of IPF for bacteremia and investigate its association with prognosis.</p><p><strong>Methods: </strong>Patients with complete blood count (CBC) on the blood culture day (D1) and the preceding day (D0) were retrospectively recruited and categorized into bacteremia and nonbacteremia groups. Immature platelet (IP) analysis, alongside CBC, was conducted. Delta IPF, defined by the absolute values of D1 minus D0 results was calculated. The ability to distinguish bacteremia from nonbacteremia patients, and the correlation with mortality were analyzed.</p><p><strong>Results: </strong>From February to December 2020, a total of 150 patients were enrolled, with 75 having bacteremia. The specificity for delta IPF ≥3.4% to predict bacteremia was 97.3% (95% confidence interval [CI]: 90.7-99.7). When delta IPF ≥3.4% combined with procalcitonin ≥0.5 (ng/mL), the sensitivity was 90.5% (95% CI: 69.6%-98.8%). Within the bacteremia group, delta IPF and the proportion of patients with delta IPF ≥1.5% were significantly higher in nonsurvival, while delta platelet levels did not. Furthermore, delta IPF ≥1.5% was independently associated with 30-day mortality (adjusted odds ratio: 3.88, 95% CI: 1.2%-11.4%; p = 0.020). The 30-day survival curve demonstrated a significant difference between patients with delta IPF ≥1.5% and those without (p < 0.001).</p><p><strong>Conclusions: </strong>Delta IPF correlates with mortality in bacteremia patients. Our findings suggest IPF not only helps detect bacteremia but also predicts prognosis in the early stage.</p>","PeriodicalId":94050,"journal":{"name":"International journal of laboratory hematology","volume":" ","pages":"51-60"},"PeriodicalIF":0.0,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142121436","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}
Pub Date : 2025-02-01Epub Date: 2024-08-07DOI: 10.1111/ijlh.14355
Shuyu E, Guilin Tang, Shaoying Li, Ellen Schlette, Sa A Wang, Jie Xu
{"title":"Anaplastic large cell lymphoma with ATIC::ALK in leukemic phase and associated with hemophagocytic lymphohistiocytosis.","authors":"Shuyu E, Guilin Tang, Shaoying Li, Ellen Schlette, Sa A Wang, Jie Xu","doi":"10.1111/ijlh.14355","DOIUrl":"10.1111/ijlh.14355","url":null,"abstract":"","PeriodicalId":94050,"journal":{"name":"International journal of laboratory hematology","volume":" ","pages":"13-14"},"PeriodicalIF":0.0,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141899244","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}
Pub Date : 2025-02-01Epub Date: 2024-11-05DOI: 10.1111/ijlh.14395
Fnu Alnoor, Alexandra Rangel, Matthew Luo, Oscar Silva, Karen M Chisholm, Dennis O'Malley, Roger Warnke, Jyoti Kumar, Robert S Ohgami
Castleman Disease (CD) is a rare lymphoproliferative disorder that can be separated into two primary forms: Unicentric Castleman disease (UCD) and multicentric Castleman disease (MCD). UCD is localized, while MCD is systemic. Though UCD generally has a favorable prognosis following surgical resection, more aggressive forms of this disease have been identified, including cases associated with dendritic and spindle cell proliferation. Genetic analysis has deepened our understanding of UCD. Despite advancements in better understanding the pathophysiology of UCD, challenges persist in the diagnosis, management, and treatment due to its rarity and heterogeneity. Here, we review current knowledge on UCD, highlighting the epidemiology, clinical presentation, diagnostic criteria, and treatment options while emphasizing the need for further research and innovation in therapeutic strategies.
{"title":"Unicentric Castleman Disease: Updates and Novel Insights Into Spindle Cell Proliferations and Aggressive Forms of a Localized Disease.","authors":"Fnu Alnoor, Alexandra Rangel, Matthew Luo, Oscar Silva, Karen M Chisholm, Dennis O'Malley, Roger Warnke, Jyoti Kumar, Robert S Ohgami","doi":"10.1111/ijlh.14395","DOIUrl":"10.1111/ijlh.14395","url":null,"abstract":"<p><p>Castleman Disease (CD) is a rare lymphoproliferative disorder that can be separated into two primary forms: Unicentric Castleman disease (UCD) and multicentric Castleman disease (MCD). UCD is localized, while MCD is systemic. Though UCD generally has a favorable prognosis following surgical resection, more aggressive forms of this disease have been identified, including cases associated with dendritic and spindle cell proliferation. Genetic analysis has deepened our understanding of UCD. Despite advancements in better understanding the pathophysiology of UCD, challenges persist in the diagnosis, management, and treatment due to its rarity and heterogeneity. Here, we review current knowledge on UCD, highlighting the epidemiology, clinical presentation, diagnostic criteria, and treatment options while emphasizing the need for further research and innovation in therapeutic strategies.</p>","PeriodicalId":94050,"journal":{"name":"International journal of laboratory hematology","volume":" ","pages":"26-35"},"PeriodicalIF":0.0,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142585426","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}
Pub Date : 2025-02-01Epub Date: 2024-09-03DOI: 10.1111/ijlh.14367
Marnix Mylemans, Nancy Boeckx, Ann Janssens, Mercedeh Tajdar, Christine Van Laer
{"title":"Fluorescence-Based Platelet Count, Blood Smear and Pre-Analytics Are Decisive in a Case of Fibrin Strand Interference Masking Severe Thrombocytopenia in an ITP Patient.","authors":"Marnix Mylemans, Nancy Boeckx, Ann Janssens, Mercedeh Tajdar, Christine Van Laer","doi":"10.1111/ijlh.14367","DOIUrl":"10.1111/ijlh.14367","url":null,"abstract":"","PeriodicalId":94050,"journal":{"name":"International journal of laboratory hematology","volume":" ","pages":"186-188"},"PeriodicalIF":0.0,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142127754","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}
Pub Date : 2025-02-01Epub Date: 2024-09-20DOI: 10.1111/ijlh.14371
Marc Trossaërt, Fabienne Genre-Volot, Valérie Horvais, Catherine Ternisien, Pierre Boisseau, Marc Fouassier, Nicolas Drillaud, Benjamin Gillet, Morgane Péré, Antoine Babuty, Emmanuelle Jeanpierre, Emmanuel de Maistre
Introduction: The entity entitled bleeding disorder of unknown cause (BDUC) qualifies individuals displaying a mild haemorrhagic profile but normal routine coagulation tests. This study was designed to evaluate whether collagen-binding assay for von Willebrand Factor (VWF) measurement (VWF:CB) could allow to diagnose VW disease in such patients.
Methods: A large screening was conducted prospectively in two University Hospitals, using the bleeding assessment tool (BAT) recommended by the International Society of Thrombosis and Hemostasis. Patients with an abnormal BAT were confirmed to have a normal complete hemostatic evaluation. A large range of VWF assays was then carried out on a new blood sample for the 68 individuals (91% women) thus identified. Of note, five VWF:CB using different types of collagen were performed, as well as a comprehensive sequencing of the VWF gene.
Results: Of this cohort, only 3 individuals (all blood group O), had a VWF:CB between 40 and 50 IU/dL. No unknown anomaly of the VWF gene was disclosed. Of note, 54% of these patients had unexplained abnormal occlusion times on PFA-200.
Conclusion: This study identified 68 cases of BDUC, after screening of a large population, indicating a low incidence. Only 3 cases were potentially confirmed as displaying moderate von Willebrand disease. VWF:CB tests were globally normal in the 65 other patients of the cohort.
简介原因不明的出血性疾病(BDUC)是指出血性症状轻微但常规凝血检测正常的患者。本研究旨在评估冯-威廉因子(VWF)胶原结合测定(VWF:CB)是否能诊断此类患者的冯-威廉因子疾病:采用国际血栓与止血学会(International Society of Thrombosis and Hemostasis)推荐的出血评估工具(BAT),在两家大学医院进行了大规模前瞻性筛查。对 BAT 异常的患者进行了全面止血评估,确认其止血功能正常。随后,对由此确定的 68 名患者(91% 为女性)的新血样进行了大量的 VWF 检测。值得注意的是,还使用不同类型的胶原蛋白进行了五次 VWF:CB 检测,并对 VWF 基因进行了全面测序:结果:在这批人中,只有 3 人(均为 O 型血型)的 VWF:CB 值在 40 至 50 IU/dL 之间。没有发现任何 VWF 基因的未知异常。值得注意的是,其中 54% 的患者在 PFA-200 检测中出现了原因不明的异常闭塞时间:这项研究在对大量人群进行筛查后发现了 68 例 BDUC,表明其发病率较低。只有 3 例可能被证实为中度 von Willebrand 病。其他 65 名患者的 VWF:CB 检测结果均正常:试验注册:ClinicalTrials.gov identifier:试验注册:ClinicalTrials.gov identifier:NCT0279220。
{"title":"Does the VWF:CB Assay Help to Diagnose von Willebrand Factor Deficiency in Patients With a Bleeding Disorder of Unknown Cause?","authors":"Marc Trossaërt, Fabienne Genre-Volot, Valérie Horvais, Catherine Ternisien, Pierre Boisseau, Marc Fouassier, Nicolas Drillaud, Benjamin Gillet, Morgane Péré, Antoine Babuty, Emmanuelle Jeanpierre, Emmanuel de Maistre","doi":"10.1111/ijlh.14371","DOIUrl":"10.1111/ijlh.14371","url":null,"abstract":"<p><strong>Introduction: </strong>The entity entitled bleeding disorder of unknown cause (BDUC) qualifies individuals displaying a mild haemorrhagic profile but normal routine coagulation tests. This study was designed to evaluate whether collagen-binding assay for von Willebrand Factor (VWF) measurement (VWF:CB) could allow to diagnose VW disease in such patients.</p><p><strong>Methods: </strong>A large screening was conducted prospectively in two University Hospitals, using the bleeding assessment tool (BAT) recommended by the International Society of Thrombosis and Hemostasis. Patients with an abnormal BAT were confirmed to have a normal complete hemostatic evaluation. A large range of VWF assays was then carried out on a new blood sample for the 68 individuals (91% women) thus identified. Of note, five VWF:CB using different types of collagen were performed, as well as a comprehensive sequencing of the VWF gene.</p><p><strong>Results: </strong>Of this cohort, only 3 individuals (all blood group O), had a VWF:CB between 40 and 50 IU/dL. No unknown anomaly of the VWF gene was disclosed. Of note, 54% of these patients had unexplained abnormal occlusion times on PFA-200.</p><p><strong>Conclusion: </strong>This study identified 68 cases of BDUC, after screening of a large population, indicating a low incidence. Only 3 cases were potentially confirmed as displaying moderate von Willebrand disease. VWF:CB tests were globally normal in the 65 other patients of the cohort.</p><p><strong>Trial registration: </strong>ClinicalTrials.gov identifier: NCT0279220.</p>","PeriodicalId":94050,"journal":{"name":"International journal of laboratory hematology","volume":" ","pages":"149-155"},"PeriodicalIF":0.0,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142304867","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}
Introduction: Ph-like ALL has gene expression profile similar to Ph-positive ALL but without the BCR::ABL1 fusion. The disease presents higher rates of severe clinical features and is associated with unfavorable outcomes. There is still no standard pipeline for molecular characterization of the disease, and no valid predictor gene panel is available worldwide.
Methods: We performed expression microarray on 25 B-cell ALL and 6 Ph-positive B-cell ALL to cluster and identify the transcriptional signature of Ph-like ALL. qRT-PCR was used to confirm the expression of candidate genes.
Results: Four out of 25 samples (16%) shared gene expression signatures related to and clustered with control Ph-positive samples. Analysis of genes differentially expressed in Ph-like B-cell ALL and evidentially functional in normal blood cell development and leukemogenesis, we selected genes as potential biomarkers for Ph-like B-cell ALL in our dataset: ADGRE2, CD9, EPHA7, FAM129C, TCL1A, and VPREB1. Those genes were filtered by Ph-like gene signatures obtained from distinct reliable data, resulting in five genes, CA6, CHN2, JAK1, JCHAIN, and PON2, selected for validation by qRT-PCR. The Ct values of genes, including CA6 (p = 0.0017), PON2 (p = 0.0210), TCL1A (p = 0.0064), and VPREB1 (p = 0.0338), were significant in Ph-like ALL. GSEA analysis identified VPREB1 as enrichment in the KRAS signaling pathway, and several genes that interact with VPREB1 were reported as critical molecules involved in the leukemogenesis of B-cell ALL.
Conclusion: In summary, we demonstrate using a gene expression microarray for classifying Ph-like B-cell ALL and highlight VPREB1 as a potential biomarker for this disease.
导言:Ph 样 ALL 的基因表达谱与 Ph 阳性 ALL 相似,但没有 BCR::ABL1 融合。该病具有较高的严重临床特征,并与不利的预后相关。目前仍没有用于该病分子特征描述的标准管道,全球范围内也没有有效的预测基因面板:方法:我们对25例B细胞ALL和6例Ph阳性B细胞ALL进行了表达芯片分析,以聚类和识别Ph样ALL的转录特征:结果:25个样本中有4个样本(16%)的基因表达特征与对照组Ph阳性样本相关并聚集在一起。通过分析在Ph样B细胞ALL中差异表达的基因以及在正常血细胞发育和白血病发生过程中具有明显功能的基因,我们在数据集中选择了一些基因作为Ph样B细胞ALL的潜在生物标记物:ADGRE2、CD9、EPHA7、FAM129C、TCL1A 和 VPREB1。根据从不同的可靠数据中获得的类 Ph 基因特征对这些基因进行筛选,最终选择了 CA6、CHN2、JAK1、JCHAIN 和 PON2 五个基因进行 qRT-PCR 验证。CA6 (p = 0.0017)、PON2 (p = 0.0210)、TCL1A (p = 0.0064) 和 VPREB1 (p = 0.0338)等基因的 Ct 值在 Ph-like ALL 中具有显著性。GSEA分析发现VPREB1在KRAS信号通路中富集,与VPREB1相互作用的几个基因被报道为参与B细胞ALL白血病发生的关键分子:总之,我们展示了利用基因表达微阵列对 Ph 型 B 细胞 ALL 进行分类的方法,并强调 VPREB1 是该疾病的潜在生物标记物。
{"title":"Application of Gene Expression Microarray for the Classification of Ph-Like B-Cell Acute Lymphoblastic Leukemia.","authors":"Nonthaya Thangrua, Teerapong Siriboonpiputtana, Budsaba Rerkamnuaychoke, Takol Chareonsirisuthigul, Veerawat Korkiatsakul, Pongpak Pongphitcha, Ekchol Mukda, Somchai Chutipongtanate, Samart Pakakasama","doi":"10.1111/ijlh.14370","DOIUrl":"10.1111/ijlh.14370","url":null,"abstract":"<p><strong>Introduction: </strong>Ph-like ALL has gene expression profile similar to Ph-positive ALL but without the BCR::ABL1 fusion. The disease presents higher rates of severe clinical features and is associated with unfavorable outcomes. There is still no standard pipeline for molecular characterization of the disease, and no valid predictor gene panel is available worldwide.</p><p><strong>Methods: </strong>We performed expression microarray on 25 B-cell ALL and 6 Ph-positive B-cell ALL to cluster and identify the transcriptional signature of Ph-like ALL. qRT-PCR was used to confirm the expression of candidate genes.</p><p><strong>Results: </strong>Four out of 25 samples (16%) shared gene expression signatures related to and clustered with control Ph-positive samples. Analysis of genes differentially expressed in Ph-like B-cell ALL and evidentially functional in normal blood cell development and leukemogenesis, we selected genes as potential biomarkers for Ph-like B-cell ALL in our dataset: ADGRE2, CD9, EPHA7, FAM129C, TCL1A, and VPREB1. Those genes were filtered by Ph-like gene signatures obtained from distinct reliable data, resulting in five genes, CA6, CHN2, JAK1, JCHAIN, and PON2, selected for validation by qRT-PCR. The Ct values of genes, including CA6 (p = 0.0017), PON2 (p = 0.0210), TCL1A (p = 0.0064), and VPREB1 (p = 0.0338), were significant in Ph-like ALL. GSEA analysis identified VPREB1 as enrichment in the KRAS signaling pathway, and several genes that interact with VPREB1 were reported as critical molecules involved in the leukemogenesis of B-cell ALL.</p><p><strong>Conclusion: </strong>In summary, we demonstrate using a gene expression microarray for classifying Ph-like B-cell ALL and highlight VPREB1 as a potential biomarker for this disease.</p>","PeriodicalId":94050,"journal":{"name":"International journal of laboratory hematology","volume":" ","pages":"130-139"},"PeriodicalIF":0.0,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142367981","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}
{"title":"Establishing reference intervals for extended red blood cell parameters with the Mindray BC-6800Plus hematology analyzer in a Chinese population.","authors":"Yanping Luo, Shuaiyan Wang, Zhuocheng Chen, Shan Lin, Sanping Guo, Hongmei Mo","doi":"10.1111/ijlh.14361","DOIUrl":"10.1111/ijlh.14361","url":null,"abstract":"","PeriodicalId":94050,"journal":{"name":"International journal of laboratory hematology","volume":" ","pages":"183-185"},"PeriodicalIF":0.0,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142038091","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}