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Laboratory Identification of Lupus Anticoagulant (LA) Using Different Activated Partial Thromboplastin Time (APTT) Assays 使用不同的活化部分凝血酶活时间(APTT)测定狼疮抗凝剂(LA)的实验室鉴定。
IF 2.3 4区 医学 Q3 HEMATOLOGY Pub Date : 2025-08-27 DOI: 10.1111/ijlh.14549
Bárbara G. Barion, Bianca Stefanello, Maria Luiza S. A. de Paula, Thaís S. Saraiva, Paula R. Villaça, Vanderson Rocha, Fernanda A. Orsi, Tania R. F. da Rocha

Introduction

The International Society of Thrombosis and Hemostasis (ISTH) guidelines suggest a three-step evaluation for the detection of lupus anticoagulant (LA), including screening, mixing, and confirmation. According to the guidelines, the LA assay based on activated partial thromboplastin time (APTT) should include an initial screening step followed by a confirmatory step that uses a higher concentration of phospholipids in either bilayer or hexagonal form. For the activator, the guidelines recommend using silica, though ellagic acid is also an option. In this context, HemosIL Silica Clotting Time (SCT, Instrumentation Laboratory) is the only assay that fully complies with the guidelines. However, there are other assays available using different reagents, such as Dade Actin FSL/FS (Siemens Healthcare Diagnostics) and PTT-LA/Staclot LA (Diagnostica Stago), and the relevance of these differences in LA detection is not known.

Methods

This study compared the performance of the three platforms.

Results

Out of 136 samples, the majority were from females (82%) with a median age of 41 years (IQR 32–50); 44 (32%) had a history of thrombosis, and 28 (21%) were on anticoagulants. PTT-LA/Staclot LA had the highest sensitivity (100%) and specificity (100%). There was an almost perfect agreement between PTT-LA/Staclot LA and Dade Actin FSL/FS (kappa 0.812). HemosIL SCT sensitivity was 100% and the specificity was 74%, which was increased to 99% by increasing the phospholipid concentration of the screening step.

Conclusion

We observed a good agreement between PTT-LA/Staclot LA and Dade Actin FSL/FS, and fair to moderate agreement with HemosIL SCT, whose performance improved with increasing phospholipid concentration. These results demonstrate that all three assays are comparable for APTT-LA detection.

简介:国际血栓与止血学会(ISTH)指南建议对狼疮抗凝剂(LA)的检测进行三步评估,包括筛选、混合和确认。根据指南,基于活化的部分凝血活素时间(APTT)的LA测定应包括一个初始筛选步骤,然后是一个确认步骤,该步骤使用更高浓度的磷脂在双层或六边形形式。对于活化剂,指南建议使用二氧化硅,尽管鞣花酸也是一种选择。在这种情况下,haemsil二氧化硅凝血时间(SCT,仪器实验室)是唯一完全符合指南的测定方法。然而,还有其他可用的检测方法,使用不同的试剂,如Dade Actin FSL/FS (Siemens Healthcare Diagnostics)和PTT-LA/Staclot LA (Diagnostica Stago),这些差异在LA检测中的相关性尚不清楚。方法:本研究比较了三种平台的性能。结果:136例样本中,大多数为女性(82%),中位年龄41岁(IQR 32-50);44例(32%)有血栓形成史,28例(21%)使用抗凝药物。PTT-LA/Staclot LA具有最高的灵敏度(100%)和特异性(100%)。PTT-LA/Staclot LA与Dade Actin FSL/FS几乎完全吻合(kappa 0.812)。haemsil SCT敏感性为100%,特异性为74%,通过增加筛选步骤的磷脂浓度,特异性可提高到99%。结论:我们观察到PTT-LA/Staclot LA与Dade Actin FSL/FS具有良好的一致性,与hematsil SCT具有中等到中等的一致性,其性能随着磷脂浓度的增加而改善。这些结果表明,这三种检测方法对APTT-LA检测具有可比性。
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引用次数: 0
Can Flow Cytometry Immunophenotyping Predict Cytogenetic Abnormalities in Acute Myeloid Leukemia? A Focus on Myelodysplasia-Related Cytogenetic Abnormalities 流式细胞术免疫分型能否预测急性髓系白血病的细胞遗传学异常?骨髓增生异常相关细胞遗传学异常的研究
IF 2.3 4区 医学 Q3 HEMATOLOGY Pub Date : 2025-08-26 DOI: 10.1111/ijlh.14546
Orathai Promsuwicha, Weerapat Owattanapanich, Supattra Kankhaw, Theera Ruchutrakool, Smith Kungwankiattichai

Introduction

The European LeukemiaNet (ELN) 2022 classification introduced significant modifications to acute myeloid leukemia (AML) categorization, including refined criteria for AML with myelodysplasia-related cytogenetic abnormalities (AML-MRC). While cytogenetic analysis is essential for a definitive diagnosis, the question remains whether flow cytometry can aid in the initial identification of this AML subgroup. This study aimed to characterize the immunophenotypic profiles of AML-MRC and validate previously reported immunophenotypic patterns of AML with t(8;21) and inv(16) using flow cytometry.

Methods

This retrospective study analyzed 911 non-acute promyelocytic leukemia (APL) AML cases. Flow cytometric immunophenotyping was performed using a comprehensive panel of 23 markers. Statistical analysis included univariate and multivariate logistic regression to identify discriminatory markers.

Results

Among 911 patients, 241 (26.5%) were classified as AML-MRC. AML-MRC patients were significantly older (mean age: 55.9 vs. 47.9 years, p < 0.001) and presented with lower WBC counts (median: 8.9 vs. 24.2 × 10^9/L, p < 0.001) compared to non-MRC cases. AML-MRC demonstrated higher expression of CD34 (75.9% vs. 57.6%, p < 0.001), CD41a (10.8% vs. 4.5%, p = 0.002) and CD235a (5.8% vs. 1.2%, p < 0.001), with CD235a showing the highest discriminatory power (OR 4.458, 95% CI 1.720–11.552). For core-binding factor AML, AML with t(8;21) exhibited characteristic expression of CD19 (46.3% vs. 9.4%, p < 0.001) and CD56 (72.5% vs. 34.5%, p < 0.001), while AML with inv(16) showed distinctive CD34 (88.9% vs. 61.7%, p = 0.004) and CD14 (59.3% vs. 18.1%, p < 0.001) expression patterns.

Conclusion

This study identifies markers that distinguish AML-MRC, including CD235a, CD41a, and CD34. This suggests that acute erythroid leukemia and acute megakaryocytic leukemia are subsets within the AML-MRC category. Additionally, the study validates previously reported immunophenotypic characteristics of AML with t(8;21) and inv(16).

欧洲白血病网(ELN) 2022分类对急性髓性白血病(AML)分类进行了重大修改,包括AML伴骨髓增生异常相关细胞遗传学异常(AML- mrc)的改进标准。虽然细胞遗传学分析对于明确的诊断是必不可少的,但流式细胞术是否可以帮助初步鉴定该AML亚群的问题仍然存在。本研究旨在利用流式细胞术表征AML- mrc的免疫表型特征,并验证先前报道的t(8;21)和inv(16) AML的免疫表型模式。方法:回顾性分析911例非急性早幼粒细胞白血病(APL) AML病例。流式细胞术免疫分型采用23个标志物的综合面板。统计分析包括单因素和多因素logistic回归,以确定歧视标记。结果:911例患者中,241例(26.5%)为AML-MRC。AML-MRC患者明显变老(平均年龄:55.9岁vs. 47.9岁),p结论:本研究确定了区分AML-MRC的标志物,包括CD235a、CD41a和CD34。这表明急性红细胞白血病和急性巨核细胞白血病是AML-MRC范畴内的亚群。此外,该研究验证了先前报道的t(8;21)和inv(16) AML的免疫表型特征。
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引用次数: 0
Fulminant Pneumococcal Bacteriemia Revealed by a Peripheral Blood Smear 外周血涂片显示暴发性肺炎球菌菌血症。
IF 2.3 4区 医学 Q3 HEMATOLOGY Pub Date : 2025-08-25 DOI: 10.1111/ijlh.14548
P. L. García-Villarroel, C. Fernández Maqueda, R. Forés Cachón
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引用次数: 0
Evaluation of 5B9 as a Calibrator or Expression of Results in Absorbance Values for the Diagnosis of Hit With a PF4/Heparin Specific Elisa 用PF4/肝素特异性Elisa评价5B9作为校正物或吸光度值对Hit诊断结果的表达
IF 2.3 4区 医学 Q3 HEMATOLOGY Pub Date : 2025-08-25 DOI: 10.1111/ijlh.14547
Claire Pouplard, Noémie Charuel, Estelle Archer, Caroline Vayne, Anne Bauters, Simon Jaouen, Philippe Savard, Laure Maucorps, Eve-Anne Guery, Yves Gruel, Jérôme Rollin

Background

Immunoassays detecting anti-PF4/H antibodies must be sensitive to exclude heparin-induced thrombocytopenia (HIT), and optical density (OD) values are useful for confirming HIT, but no calibration is currently available.

Objectives

To study the impact of OD values on the performance of the Asserachrom HPIA IgG in a cohort of patients with suspected HIT, and the value of a calibration performed with 5B9, a HIT monoclonal antibody.

Methods

The HPIA IgG was performed in 170 patients with a high or intermediate probability of HIT. Results were expressed in OD450 or ‘5B9 equivalent’ units, using a calibration done with 5B9. HIT was confirmed when HPIA and SRA/PF4 tests were positive.

Results

HIT was excluded in 97 cases because HPIA and SRA/PF4 were negative. The HPIA was positive in 73 cases and HIT confirmed in 43 cases (SRA/PF4+). Applying an OD threshold of 1.05, the NPV and PPV of the test were 98% and 83%, respectively. Calibration of HPIA with 5B9 did not improve its performance, since similar AUC values (ROC curves) were obtained whether results were expressed in OD values or in equivalent units of 5B9. Bayesian analysis showed that in patients with an intermediate pre-test probability of HIT, the post-test probability equalled 1% when OD was less than 1, and 100% when OD was over 2.

Conclusion

5B9 as a calibrator failed to improve the performance of HPIA, but this assay can reliably exclude (when negative) or confirm HIT (when OD > 2), without requiring a functional assay.

背景:检测抗pf4 /H抗体的免疫测定必须敏感,以排除肝素诱导的血小板减少症(HIT),光密度(OD)值可用于确认HIT,但目前尚无校准方法。目的:研究OD值对可疑HIT患者中Asserachrom HPIA IgG检测性能的影响,以及HIT单克隆抗体5B9的校准值。方法:对170例中高概率HIT患者进行HPIA IgG检测。结果以OD450或“5B9当量”单位表示,使用5B9进行校准。当HPIA和SRA/PF4试验阳性时,证实HIT。结果:97例HPIA、SRA/PF4阴性,排除HIT。HPIA阳性73例,HIT确诊43例(SRA/PF4+)。OD阈值为1.05,NPV和PPV分别为98%和83%。用5B9校准HPIA并没有改善其性能,因为无论结果以OD值表示还是以5B9的等效单位表示,得到的AUC值(ROC曲线)都是相似的。贝叶斯分析显示,在HIT前测概率中等的患者中,OD小于1时,后测概率为1%,OD大于2时,后测概率为100%。结论:5B9作为校准剂不能提高HPIA的性能,但该方法可以可靠地排除(当阴性时)或确认HIT(当OD为> 2时),而不需要进行功能分析。
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引用次数: 0
Compound Heterozygous Hb Milledgeville With −α4.2 Thalassemia—A Rare and First Reported Cause of Primary Erythrocytosis in an Indian Family 复合杂合Hb Milledgeville伴-α4.2地中海贫血——一种罕见且首次报道的印度家庭原发性红血病病因。
IF 2.3 4区 医学 Q3 HEMATOLOGY Pub Date : 2025-08-24 DOI: 10.1111/ijlh.14544
Richa Chauhan, Vandana Puri, Shreyam Acharya, Jasmita Dass, Ravi Ranjan, Prashant Sharma, Ganesh Kumar Viswanathan, Mukul Aggarwal, Pradeep Kumar, Rishi Dhawan, Tulika Seth, Manoranjan Mahapatra

Recent review collated 22 rare and novel alpha globin gene variants amongst the Indian population published in the literature in the last 52 years. We report another rare high-oxygen affinity alpha-globin variant hemoglobinopathy in a compound heterozygous state with α+ thalassemia. The patient, a 42-year-old male, came for evaluation of JAK2 p.V617F negative erythrocytosis requiring multiple phlebotomies in the last 4–5 years. On laboratory investigations for high-oxygen affinity hemoglobinopathy, he was found to have an unknown peak eluting as a left shoulder hump of the Adult hemoglobin (HbA0) in Cation exchange High-Performance Liquid Chromatography (CE-Hb-HPLC). His family study revealed the variant hemoglobinopathy coexisting with a single alpha-globin deletion in the mother, sibling, and two children. Next-generation sequencing (NGS), Gap Polymerase chain reaction (GAP-PCR), and multiplex ligation probe amplification (MLPA) on the extracted DNA of the index case showed compound heterozygous state for Hb Milledgeville and −α4.2 thalassemia. This is the first report of a rare high-oxygen-affinity alpha hemoglobin variant Hb Milledgeville from India.

最近的综述整理了过去52年中在印度人口中发表的22种罕见和新颖的α -珠蛋白基因变异。我们报告另一个罕见的高氧亲和α -珠蛋白变异血红蛋白病在复合杂合状态与α+地中海贫血。患者,42岁男性,在过去的4-5年里,为了评估JAK2 p.V617F阴性红细胞,需要多次抽血。在高氧亲和性血红蛋白病的实验室调查中,在阳离子交换高效液相色谱(CE-Hb-HPLC)中,发现他有一个未知的峰洗脱,作为成人血红蛋白(HbA0)的左肩驼峰。他的家庭研究显示,在母亲、兄弟姐妹和两个孩子中,变异型血红蛋白病与单个α -珠蛋白缺失共存。新一代测序(NGS)、Gap聚合酶链反应(Gap - pcr)和多重连接探针扩增(MLPA)对该病例提取的DNA进行检测,结果显示该病例的Hb Milledgeville和-α4.2地中海贫血呈复合杂合状态。这是来自印度的一种罕见的高氧亲和α血红蛋白变异Hb Milledgeville的第一份报告。
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引用次数: 0
Reticulocyte Count Interference at the Onset of Acute Myeloid Leukemia 急性髓系白血病发病时网织红细胞计数的干扰。
IF 2.3 4区 医学 Q3 HEMATOLOGY Pub Date : 2025-08-20 DOI: 10.1111/ijlh.14545
María del Mar Gutiérrez-Hernández, Guillermo Ramil, Nuria González-Álvarez, Paula San-José
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引用次数: 0
Aggressive T-Cell Large Granular Lymphocyte Leukemia With the Unusual Double-Negative CD4−/CD8−/TCRαβ+ Phenotype; Report of Two Cases and Review of the Literature 侵袭性t细胞大颗粒淋巴细胞白血病伴异常双阴性CD4-/CD8-/TCRαβ+表型两例报告及文献复习。
IF 2.3 4区 医学 Q3 HEMATOLOGY Pub Date : 2025-08-20 DOI: 10.1111/ijlh.14540
Angeliki Kotsiafti, Nikolaos J. Tsagarakis, Chrissa M. Vadikolia, Dimitrios Maltezas, Christina Karela, Georgios Oudatzis, Panagiotis Repousis, Paraskevi Vasileiou, Georgios Paterakis
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引用次数: 0
An Atypical Case of Burkitt Lymphoma With MYC Gain and Cryptic IGH::MYC Rearrangement Detected by Rapid Nanopore Sequencing 快速纳米孔测序检测MYC增益和隐性IGH::MYC重排的非典型伯基特淋巴瘤1例。
IF 2.3 4区 医学 Q3 HEMATOLOGY Pub Date : 2025-08-20 DOI: 10.1111/ijlh.14542
Ka Ngai Lau, Tsz Fung Wong, Lawrence Lap Chi Tsui, Ka Wai Wong, Yuen Ting Sin, Coty Hing Yau Cheung, Eleanor Koon Chun Hui, Joyce Sin Cheung, Alice Ching Ching Wong, Sze Fai Yip
{"title":"An Atypical Case of Burkitt Lymphoma With MYC Gain and Cryptic IGH::MYC Rearrangement Detected by Rapid Nanopore Sequencing","authors":"Ka Ngai Lau,&nbsp;Tsz Fung Wong,&nbsp;Lawrence Lap Chi Tsui,&nbsp;Ka Wai Wong,&nbsp;Yuen Ting Sin,&nbsp;Coty Hing Yau Cheung,&nbsp;Eleanor Koon Chun Hui,&nbsp;Joyce Sin Cheung,&nbsp;Alice Ching Ching Wong,&nbsp;Sze Fai Yip","doi":"10.1111/ijlh.14542","DOIUrl":"10.1111/ijlh.14542","url":null,"abstract":"","PeriodicalId":14120,"journal":{"name":"International Journal of Laboratory Hematology","volume":"47 6","pages":"1206-1210"},"PeriodicalIF":2.3,"publicationDate":"2025-08-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144983454","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Exploring Cell Population Data for the Diagnosis and Assessment of Severity in Sepsis: A Preliminary Report 探索细胞群数据诊断和评估脓毒症的严重程度:初步报告。
IF 2.3 4区 医学 Q3 HEMATOLOGY Pub Date : 2025-08-18 DOI: 10.1111/ijlh.14539
Priyanka Mishra, Pratik Thosani, Mrinal Patra, Preeti Tripathi, Mallikarjun Dube, Brajesh Singh

Background

Cell Population Data (CPD), derived from next-generation hematology analyzers, is emerging as a promising tool for diagnosis of early sepsis. This preliminary case–control study assessed the diagnostic utility of CPD and its association with sequential organ failure assessment (SOFA) scores and procalcitonin levels in sepsis patients.

Methods

Seventy-two sepsis patients and 72 age- and sex-matched non-septic controls were enrolled. CPD parameters were measured using a Sysmex XN-1000 analyzer. Univariate and multivariate analyses, including PCA, PLS-DA, and OPLS-DA, were used to distinguish between groups. ROC analysis evaluated diagnostic performance. Spearman's rank correlation assessed associations between CPD, SOFA scores, and procalcitonin.

Results

Several CPD parameters—LY-X, LY-Z, MO-X, MO-Y, NE-WX, NE-WY, NE-WZ, LY-WX, LY-WY, and LY-WZ—were significantly elevated in sepsis. Multivariate analysis identified MO-X, MO-WY, LY-X, and LY-Z as strong discriminators (VIP > 1.15). ROC analysis showed MO-X (> 119.6) had 95.83% sensitivity, 73.61% specificity (AUC 0.876), and IG% (> 0.6) had 83.33% sensitivity, 80.56% specificity (AUC 0.880). IG count (> 40/μL) showed 81.94% sensitivity, 81.32% specificity (AUC 0.859); LY-X (> 79.3) had 93.06% sensitivity, 48.61% specificity (AUC 0.685); and MO-WY (> 752) had 84.72% sensitivity, 45.83% specificity (AUC 0.597). SOFA score correlated with MO-X (r = 0.40, p = 0.007), LY-X (r = 0.40, p = 0.008) and LY-Z (r = 0.39, p = 0.009), while procalcitonin correlated with MO-X (r = 0.30, p = 0.03) and HFLC (r = 0.40, p = 0.005).

Conclusion

CPD is a promising, cost-effective biomarker for diagnosis and severity assessment of sepsis, but inter-equipment variability and current “research use only” status warrant further clinical validation.

背景:细胞群数据(CPD)源于下一代血液学分析仪,正在成为早期败血症诊断的一种有前途的工具。这项初步的病例对照研究评估了CPD的诊断效用及其与脓毒症患者序贯器官衰竭评估(SOFA)评分和降钙素原水平的关系。方法:纳入72例败血症患者和72例年龄和性别匹配的非败血症对照组。CPD参数测量采用Sysmex XN-1000分析仪。单因素和多因素分析,包括PCA、PLS-DA和OPLS-DA,用于组间区分。ROC分析评估诊断表现。Spearman等级相关性评估了CPD、SOFA评分和降钙素原之间的关联。结果:脓毒症患者CPD参数ly - x、LY-Z、MO-X、MO-Y、NE-WX、NE-WZ、LY-WX、LY-WY、ly - wz显著升高。多变量分析发现MO-X、MO-WY、LY-X和LY-Z是强鉴别因子(VIP > 1.15)。ROC分析显示,MO-X(> 119.6)的敏感性为95.83%,特异性为73.61% (AUC 0.876), IG%(> 0.6)的敏感性为83.33%,特异性为80.56% (AUC 0.880)。IG计数(bbb40 /μL)敏感性为81.94%,特异性为81.32% (AUC 0.859);LY-X(> 79.3)敏感性93.06%,特异性48.61% (AUC 0.685);MO-WY(> 752)敏感性84.72%,特异性45.83% (AUC 0.597)。SOFA评分与MO-X (r = 0.40, p = 0.007)、LY-X (r = 0.40, p = 0.008)、LY-Z (r = 0.39, p = 0.009)相关,降钙素原与MO-X (r = 0.30, p = 0.03)、HFLC (r = 0.40, p = 0.005)相关。结论:CPD是一种有前景的、具有成本效益的脓毒症诊断和严重程度评估的生物标志物,但设备间的差异和目前“仅用于研究”的状态需要进一步的临床验证。
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引用次数: 0
Breaking Free From MCHC Interferences? French-Speaking Cellular Haematology Group (GFHC) Review of Causes, Rising Trends and Practical Solutions 摆脱母婴健康中心的干扰?法语细胞血液病小组(GFHC)的原因,上升趋势和实际解决方案的审查。
IF 2.3 4区 医学 Q3 HEMATOLOGY Pub Date : 2025-08-17 DOI: 10.1111/ijlh.14536
Sandrine Girard, Yaël Berda-Haddad, Chantal Brouzes, Bouchra Badaoui, Agathe Boussaroque, Alexandre Janel, Bernard Chatelain, Véronique Baccini

Mean corpuscular haemoglobin concentration (MCHC) is determined by the ratio of haemoglobin concentration to haematocrit. Managing increased MCHC presents significant challenges, mainly due to variations in analytical methods and pathophysiological conditions. Depending on the haematological analyser (HA), MCHC can be measured directly or calculated. It is important that all people involved in hematocytometry must identify and correct artefacts to ensure accurate erythrocyte parameters. In order to harmonise and standardise haematology practices in all laboratories, the French-speaking Cellular Haematology Group (GFHC) has reviewed the interferences and pathophysiological situations that could increase MCHC, and the advice on how to manage cases of elevated MCHC. We will review current techniques, such as impedance and optical methods, for accurate determination of MCHC. We will also examine the interferences that can artificially increase MCHC; and the pathophysiological conditions responsible for such increases. Finally, we will present guidelines for the management of elevated MCHC, including strategies to bypass interferences and determine which erythrocyte parameters can be reliably reported, as well as the acceptable MCHC values for various pathophysiological variations.

红细胞平均血红蛋白浓度(MCHC)由血红蛋白浓度与红细胞压积的比值决定。管理增加的MCHC提出了重大挑战,主要是由于分析方法和病理生理条件的变化。根据血液学分析仪(HA), MCHC可以直接测量或计算。重要的是,所有参与血细胞测定的人必须识别和纠正伪影,以确保准确的红细胞参数。为了协调和标准化所有实验室的血液学操作,法语细胞血液学小组(GFHC)审查了可能增加母婴健康的干扰和病理生理情况,以及如何管理母婴健康升高病例的建议。我们将回顾当前的技术,如阻抗和光学方法,以准确测定MCHC。我们还将研究人为增加MCHC的干扰;以及导致这种增加的病理生理条件。最后,我们将提出MCHC升高的管理指南,包括绕过干扰的策略,确定哪些红细胞参数可以可靠地报告,以及各种病理生理变化的可接受的MCHC值。
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引用次数: 0
期刊
International Journal of Laboratory Hematology
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