International journal of laboratory hematology最新文献

{"title":"Diagnosing Systemic Mastocytosis: State of the Art.","authors":"Anton Rets, Tracy I George","doi":"10.1111/ijlh.70011","DOIUrl":"https://doi.org/10.1111/ijlh.70011","url":null,"abstract":"<p><p>With the advent of effective multikinase and selective tyrosine kinase inhibitors in systemic mastocytosis, diagnosing this rare disease has been critical to improving patient morbidity and mortality. This state-of-the-art review interprets the international diagnostic criteria, including differences between the WHO 5th edition classification and the International Consensus Classification. Subclassification of systemic mastocytosis is critical for correct therapeutic strategies, and diagnostic difficulties are described for the practicing pathologist. Morphologic mimics, which require alternative treatment, are discussed.</p>","PeriodicalId":94050,"journal":{"name":"International journal of laboratory hematology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145245978","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":"Sysmex Cell Population Data for Diagnosing Infection in Patients With Suspected Sepsis in the Emergency Department.","authors":"Kristin E Wickstrøm, Aleksander Rygh Holten, Christian Prebensen, Alvaro Köhn-Luque, Valeria Vitelli, Erik Koldberg Amundsen","doi":"10.1111/ijlh.70008","DOIUrl":"https://doi.org/10.1111/ijlh.70008","url":null,"abstract":"<p><strong>Objectives: </strong>Early diagnosis of suspected sepsis is crucial to improve patient survival. Cell population (CP) data, a set of leucocyte research parameters from hematology instruments, has a potential as markers for infection. The aim of this study was to investigate the diagnostic accuracy for infection of CP variables from Sysmex XN instruments in patients with suspected sepsis in the emergency department (ED).</p><p><strong>Methods: </strong>Adult patients with suspected sepsis in the ED were included. CP variables, C-reactive protein (CRP), and post hoc assessments of infection were recorded. Logistic regression and machine learning methods were used to develop multivariable models, which were evaluated by area under the receiver operating curve (AUC) and calibration plots.</p><p><strong>Results: </strong>The development cohort and the validation cohort consisted of 600 and 656 patients, respectively. Univariate analyses revealed that complexity in monocytes (MO-X); AUC of 0.78 (0.74, 0.82), reactivity intensity of neutrophils (NEUT-RI); 0.72 (0.67, 0.76), and CRP 0.87 (0.84, 0.90) had the highest diagnostic accuracy for infection. A final multivariable model (the optimal model) using Multilayer perceptron (MLP), including MO-X, NEUT-RI, monocyte size (MO-Z), and neutrophil size (NE-FSC), had an AUC of 0.86 (0.85, 0.87) in the development cohort and 0.78 (0.74, 0.82) in the validation cohort with reasonable calibration. Including CRP in this model further improved accuracy and calibration.</p><p><strong>Conclusions: </strong>Sysmex CP variables may help diagnose infections in the ED. However, the lack of well-described calibration procedures and quality assurance for non-IVD approved CP variables is an impediment to clinical implementation.</p>","PeriodicalId":94050,"journal":{"name":"International journal of laboratory hematology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-10-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145215066","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":"Atypical Lymphocytosis Induced by T Cell-Engaging Therapy in Patients With Hematological Malignancies.","authors":"Tae-Shin Kim, Hyun Kyung Kim, Seon Young Kim, Yoon Hwan Chang","doi":"10.1111/ijlh.70007","DOIUrl":"https://doi.org/10.1111/ijlh.70007","url":null,"abstract":"<p><strong>Introduction: </strong>Atypical lymphocytes (ALYs) are activated lymphocytes with distinct morphological characteristics, often observed in various infections, autoimmune diseases, drug reactions, and malignancies. Their appearance may resemble leukemic or lymphoma cells, making it essential to differentiate ALYs, particularly in patients with hematological malignancies. With the advent of T-cell engagers (TCEs), a novel class of immuno-oncology drugs, this study aimed to investigate their effect on peripheral blood profiles, including ALYs.</p><p><strong>Methods: </strong>We retrospectively analyzed complete blood count (CBC) data and peripheral blood morphology from 28 patients enrolled in clinical trials of various TCEs targeting multiple myeloma and B-cell lymphomas. The drugs studied included cevostamab, linvoseltamab, glofitamab, teclistamab, talquetamab, elranatamab, and epcoritamab.</p><p><strong>Results: </strong>A transient increase in ALYs was observed in 11 of the 28 patients treated with TCEs. This was confirmed by changes in cell morphology and flow cytometric parameters obtained from the CBC analyzer. ALY elevation appeared to be influenced by drug type, administration route, and combination therapies. In addition, a sudden and transient decrease in both monocytes and lymphocytes was noted in peripheral blood following cevostamab treatment.</p><p><strong>Conclusion: </strong>The observed increase in ALYs likely reflects immune activation induced by TCEs. Understanding ALY dynamics during TCE treatment is crucial for clinicians and pathologists when interpreting patient test results. Furthermore, ALY testing may serve as a potential marker for predicting the effectiveness of TCE therapies.</p>","PeriodicalId":94050,"journal":{"name":"International journal of laboratory hematology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-09-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145180844","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":"ADAMTS13 Activity Testing: Evaluation of Two Automated Platforms for Diagnosis and Follow-Up of Thrombotic Thrombocytopenic Purpura.","authors":"Margot Cornette, Katrien M J Devreese","doi":"10.1111/ijlh.70004","DOIUrl":"https://doi.org/10.1111/ijlh.70004","url":null,"abstract":"<p><strong>Introduction: </strong>Thrombotic thrombocytopenic purpura (TTP) is a rare but life-threatening thrombotic microangiopathy characterized by a severe deficiency of ADAMTS13 activity, typically defined as less than 10 IU/dL. Rapid and reliable measurement of ADAMTS13 activity is crucial for both timely diagnosis and effective long-term patient monitoring. While ELISA-based methods are widely used, automated assays offer potential advantages in terms of turnaround time and standardization. This study evaluates two automated ADAMTS13 activity assays, HemosIL AcuStar ADAMTS13 Activity Assay (Werfen) and TECHNOFLUOR ADAMTS13 Activity (Technoclone), in comparison to the TECHNOZYM ADAMTS13 Activity ELISA (Technoclone).</p><p><strong>Methods: </strong>ADAMTS13 activity was measured in 100 patient samples using three assays: TECHNOZYM ADAMTS13 Activity ELISA, HemosIL AcuStar ADAMTS13 Activity, and TECHNOFLUOR ADAMTS13 Activity. Analytical performance and method comparison were assessed. Additionally, calibration against the WHO standard was evaluated.</p><p><strong>Results: </strong>Both automated assays demonstrated high precision, with lower coefficients of variation (CV) compared to the ELISA. The HemosIL assay showed CVs ranging from 4.6% to 7.1%, while the TECHNOFLUOR assay exhibited CVs between 3.0% and 3.3%. ADAMTS13 activity measurements obtained with ELISA were significantly higher than those from both automated assays, which showed no statistically significant difference between them. Both automated assays achieved a sensitivity of 100%, with one false-positive TTP classification. The HemosIL assay showed the greatest bias between WHO and manufacturer-based calibrations.</p><p><strong>Conclusion: </strong>The automated systems exhibit strong performance and rapid turnaround times, making them well-suited for routine ADAMTS13 determination. Standardization and calibration against international benchmarks, such as the WHO standard, is essential to minimize inter-assay variability and ensure reliable clinical interpretation.</p>","PeriodicalId":94050,"journal":{"name":"International journal of laboratory hematology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145152421","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":"Transcriptome Analysis Identifies Functional and Prognostic Hypoxia-Associated Genes in Multiple Myeloma.","authors":"Lijia Hou, Jing Zhang, Qian Ran, Zhongjun Li, Maoshan Chen","doi":"10.1111/ijlh.70009","DOIUrl":"https://doi.org/10.1111/ijlh.70009","url":null,"abstract":"<p><strong>Introduction: </strong>Multiple myeloma (MM) is an incurable clonal B-cell malignancy characterized by the accumulation of neoplastic plasma cells in the bone marrow (BM). Many pieces of evidence indicate that hypoxia promotes MM progression, but the underlying mechanisms are not well known.</p><p><strong>Methods: </strong>We analyzed gene expression profiles of 3 MM cell lines under hypoxia and the MMRF CoMMpass project. We validated the expression patterns of hypoxia-associated genes (HAGs) in CD138+ BM cells from MM patients at different stages. Single-cell RNA sequencing data were used to analyze the performance of HAGs in the BM microenvironment.</p><p><strong>Results: </strong>We identified 17 HAGs differentially expressed in three MM cell lines under hypoxia. While in the MMRF project, we identified 92 differentially expressed HAGs in newly diagnosed MM patients. MM cell lines and the MMRF project shared 9 HAGs, including ADM, BNIP3L, EGLN1, FAM162A, HMOX1, PDK1, PLOD1, STAT5B, and TFRC. Notably, 8 of them were significantly associated with the overall survival of MM patients, and 6 were significantly associated with the MM patient survival in the first year after diagnosis. Then, hypoxia pressure scores calculated using these genes displayed significant differences between MM patients and healthy individuals. Further, we validated the expression patterns of HAGs using another cohort data and performed qRT-PCR using our own samples, and the results confirmed severe hypoxia existed in plasma cells and other cell types of the BM microenvironment of MM patients compared to healthy individuals.</p><p><strong>Conclusion: </strong>Taken together, our findings may contribute to the treatment and prognosis prediction of MM patients.</p>","PeriodicalId":94050,"journal":{"name":"International journal of laboratory hematology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145180002","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":"Predictive Performance of Neutrophil Parameter for Neutrophil Recovery From Prolonged Neutropenia.","authors":"Kazutoshi Ebisawa, Masaki Tanibuchi, Kunitoshi Fukazawa, Takahiro Takeuchi","doi":"10.1111/ijlh.70002","DOIUrl":"https://doi.org/10.1111/ijlh.70002","url":null,"abstract":"<p><strong>Introduction: </strong>Infectious complications occurring in patients with neutropenia remained one of the major causes of mortality in the treatment of hematologic malignancies. Rapid and exact prediction of neutrophil recovery would be helpful for timely and appropriate management of these complications.</p><p><strong>Objective: </strong>The aim of this study was to evaluate the performance of neutrophil parameters, especially NE-WX, for predicting the timing of neutrophil recovery.</p><p><strong>Materials and methods: </strong>We retrospectively reviewed patients who were hospitalized in our department and analyzed absolute neutrophil counts (ANC), NE-WX, and monocyte counts.</p><p><strong>Results: </strong>Median NE-WX and ANC of 2428 blood tests for 154 patients was 334 (Interquartile range (IQR): 312-386) and 3.124 × 10⁹/L (IQR: 1.165-6.136 × 10⁹/L). When NE-WX ranged from 0 to 100 or from 101 to 200, median ANCs were 0.02 × 10⁹/L (IQR: 0.01-0.02 × 10⁹/L) or 0.03 × 10⁶/L (IQR: 0.02-0.623 × 10⁹/L), respectively. Such extreme low NE-WX levels were observed only in patients with very severe neutropenia, which was defined as ANC of less than 0.2 × 10⁹/L. Furthermore, analyses of chronological changes of NE-WX and ANC elucidated that although the behaviors of these parameters were basically parallel, recovery of ANC from neutropenia was preceded by the recovery of NE-WX (15 vs. 18 days; p < 0.01).</p><p><strong>Conclusion: </strong>Our analyses revealed that low NE-WX levels were observed only in patients with very severe neutropenia, and in these cases, increases in NE-WX levels might predict the timing of neutrophil recovery.</p>","PeriodicalId":94050,"journal":{"name":"International journal of laboratory hematology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145152373","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":"Comparison of Erythrocyte Indices With A Newly Developed Mean Corpuscular Hemoglobin Concentration Based Formula for Differentiating β-Thalassemia Minor From Other Microcytic Hypochromic Anemia.","authors":"Yehezkiel Yonathan, Nina Susana Dewi, Agnes Rengga Indrati","doi":"10.1111/ijlh.14558","DOIUrl":"https://doi.org/10.1111/ijlh.14558","url":null,"abstract":"<p><strong>Background: </strong>Erythrocyte indices are measuring tools for differentiating beta thalassemia (β-thalassemia) minor from other microcytic hypochromic anemias using routine hematological parameters. Although indices such as the Mentzer Index, Shine and Lal (S&L), Green and King, Ehsani, and Srivastava are widely applied, diagnostic accuracy remains limited when compared to hemoglobin electrophoresis, which serves as the gold standard.</p><p><strong>Objective: </strong>To compare the performance of two newly developed erythrocyte formulas for identifying β-thalassemia minor in comparison with five commonly used indices.</p><p><strong>Methods: </strong>A cross-sectional study was conducted on 54 males and 214 female subjects with microcytic hypochromic anemia. Two new formulas were developed based on Mean Corpuscular Hemoglobin Concentration (MCHC), a parameter available in simple hematologic analyzers. The first formula was derived using the binary logistic regression method (21.33 + 0.9MCH-1.3MCHC), while the second was constructed as a ratio of Mean Corpuscular Volume (MCV) and MCHC (MCV/MCHC² × 100). Diagnostic performance was compared against other widely used erythrocyte indices.</p><p><strong>Results: </strong>Shine and Lal Index had the highest sensitivity compared to all erythrocyte indices (98.65%). However, the newly developed formulas had the highest specificity (92.50% and 90.01%), positive predictive value (PPV) (93.48% and 91.55%), negative predictive value (NPV) (85.38% and 85.71%), accuracy (89.55% and 88.81%), Youden Index (79.66% and 77.85%), and Area Under the Curve (AUC) (0.932 and 0.934) compared to all erythrocyte indices.</p><p><strong>Conclusion: </strong>The two new MCHC-based formulas had the best diagnostic performance compared with other erythrocyte indices, while Shine and Lal Index had the highest sensitivity.</p>","PeriodicalId":94050,"journal":{"name":"International journal of laboratory hematology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145139803","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":"Pyruvate Kinase Deficiency: An Underdiagnosed Cause of Severe Hemolytic Anemia in Iranian Population: Insights From Whole Exome Sequencing of Four Families and Screening of a Population-Specific Database.","authors":"Milad Rafat, Yassin Bouraqi, Jafar Mehrabi Sisakht, Azita Azarkeivan, Hossein Najmabadi, Maryam Neishabury","doi":"10.1111/ijlh.70003","DOIUrl":"https://doi.org/10.1111/ijlh.70003","url":null,"abstract":"<p><strong>Introduction: </strong>Pyruvate kinase deficiency (PKD) is a potentially underdiagnosed cause of chronic hemolytic anemia worldwide and remains understudied in certain populations, including Iran. Here, we describe PKD diagnoses in seven Iranian patients from four consanguineous families. Additionally, we present evidence from a population-specific database that supports the widespread prevalence of this disease in the country.</p><p><strong>Materials and methods: </strong>Whole exome sequencing (WES) was performed on probands presenting with hyperbilirubinemia and severe hemolytic anemia, who were referred to us by specialists after exclusion of hemoglobinopathies, autoimmune hemolytic anemia, and G6PD deficiency, with no definitive diagnosis established. Family studies were conducted using Sanger sequencing. The Iranome database was screened for pathogenic PKLR mutations.</p><p><strong>Results: </strong>Three distinct PKLR variants were identified in these families: a known pathogenic variant, a novel likely pathogenic variant, and a variant of unknown significance that had previously been reported only once in the compound heterozygous state in two Iranian siblings. Also, a retrospective analysis of the Iranome database revealed that 21 individuals from various Iranian ethnic groups, out of 1200, carried one of six distinct variants classified as pathogenic or likely pathogenic based on the American College of Medical Genetics and Genomics (ACMG) guidelines.</p><p><strong>Discussion and conclusion: </strong>This study provides new insights into the genetic and ethnic diversity of PKD and its prevalence in Iran. It also highlights critical risk factors for PKD in the Iranian population, including clinical and molecular diagnostic challenges, the prevalence of consanguineous marriages, and low public awareness regarding the risks of genetic disorders.</p>","PeriodicalId":94050,"journal":{"name":"International journal of laboratory hematology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145082788","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":"Prolonged Heparin Re-Exposure in a Patient With Previous Heparin-Induced Thrombocytopenia (HIT).","authors":"Christopher Pleyer, Gina Ney, Khanh Nghiem, Shelley Kalsi, Charles Bolan, Katherine R Calvo, Michelly K Sampaio de Melo, Douglas R Stewart, James Maiarana, Arjun Kanuri, Laura Tosi, Jo-Ann I Sheppard, Theodore E Warkentin","doi":"10.1111/ijlh.14552","DOIUrl":"https://doi.org/10.1111/ijlh.14552","url":null,"abstract":"","PeriodicalId":94050,"journal":{"name":"International journal of laboratory hematology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-09-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145077024","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":"The Extended Complete Blood Count Requires a Transition From the Abbreviation of Indices to Their Digital Hierarchy and to the Graphical Representation of Results.","authors":"Valeri P Maltsev","doi":"10.1111/ijlh.70006","DOIUrl":"https://doi.org/10.1111/ijlh.70006","url":null,"abstract":"","PeriodicalId":94050,"journal":{"name":"International journal of laboratory hematology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145071388","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}