Acta Haematologica最新文献

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DRESS Is Not a Rare Complication during the Initial Treatment of Newly Diagnosed Multiple Myeloma: The Experience of Two Medical Institutes.
IF 1.7 4区 医学
Acta Haematologica Pub Date : 2025-01-27 DOI: 10.1159/000543779
Jiao Yang, Qingqing Hu, Jiaqing Lu, Jian Wang, Die Wu, Xiaoming Fei, Lixia Wang, Xianqiu Yu, Yu Tang
{"title":"DRESS Is Not a Rare Complication during the Initial Treatment of Newly Diagnosed Multiple Myeloma: The Experience of Two Medical Institutes.","authors":"Jiao Yang, Qingqing Hu, Jiaqing Lu, Jian Wang, Die Wu, Xiaoming Fei, Lixia Wang, Xianqiu Yu, Yu Tang","doi":"10.1159/000543779","DOIUrl":"10.1159/000543779","url":null,"abstract":"<p><strong>Introduction: </strong>Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe hypersensitivity reaction rarely documented in patients with multiple myeloma (MM).</p><p><strong>Methods: </strong>In our retrospective study of 108 newly diagnosed MM (NDMM) patients from January 2021 to October 2023, we identified 4 cases of DRESS. The clinical characteristics such as clinical manifestations, laboratory results, treatment, and outcome were analyzed.</p><p><strong>Results: </strong>These patients presented with fever, persistent and recurrent, along with a widespread red rash characterized by diffuse erythematous macules or papules accompanied by intense itching, desquamation, and other dermatological manifestations. Multiorgan involvement was common, including hepatic impairment, acute kidney injury, and type I respiratory failure, alongside pleural effusion and multiple lymphadenopathy. Laboratory findings revealed elevated eosinophil counts, often exceeding 1.5 × 109/L, abnormal liver function tests, acute kidney injury, and inflammatory markers. Anti-MM treatment was promptly suspended, and all patients received corticosteroid therapy. Outcomes varied, with 1 patient succumbing to myeloma progression, another to multiorgan failure, while the remaining 2 patients survived.</p><p><strong>Conclusion: </strong>In NDMM patients undergoing induction therapy, occurrences of DRESS are infrequent but noteworthy, with an incidence higher than observed in the general population. It presents with significant morbidity and mortality, highlighting the crucial need for early recognition and management.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"1-9"},"PeriodicalIF":1.7,"publicationDate":"2025-01-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143050982","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
A Prognostic Survival Model Based on Endocrine-Related Gene Expression in Acute Myelogenous Leukemia. 急性髓性白血病中基于内分泌相关基因表达的预后生存模型。
IF 1.7 4区 医学
Acta Haematologica Pub Date : 2025-01-14 DOI: 10.1159/000543272
Weiran Lv, Yun Wang, Fang Hu, Hanying Huang, Yingying Cui, Yuanbin Song, Lezong Chen, Bingyi Wu, Yang Liang
{"title":"A Prognostic Survival Model Based on Endocrine-Related Gene Expression in Acute Myelogenous Leukemia.","authors":"Weiran Lv, Yun Wang, Fang Hu, Hanying Huang, Yingying Cui, Yuanbin Song, Lezong Chen, Bingyi Wu, Yang Liang","doi":"10.1159/000543272","DOIUrl":"10.1159/000543272","url":null,"abstract":"<p><strong>Introduction: </strong>Accurate prediction of survival in patients with acute myelogenous leukemia (AML) is challenging. Therefore, we developed a predictive survival model using endocrine-related gene expression to identify an endocrine signature for accurate stratification of AML prognosis.</p><p><strong>Methods: </strong>RNA matrices and clinical data for AML were downloaded from a training dataset (Gene Expression Omnibus) and two validation datasets (the Cancer Genome Atlas and Therapeutically Applicable Research to Generate Effective Treatments).</p><p><strong>Results: </strong>In relation to the survival outcome, a risk model was constructed by incorporating seven endocrine-related genes. The model exhibited favorable predictive efficacy in estimating 5-year survival rates, as demonstrated by both the training and validation cohorts. Multivariable analysis revealed that the endocrine signature demonstrated autonomous prognostic significance in the aforementioned cohorts. Prediction accuracy for 5-year overall survival increased using a nomogram combining endocrine risk score and classical prognostic factors compared with using classical prognostic factors alone. The model predictions were confirmed using AML cell lines.</p><p><strong>Conclusion: </strong>The endocrine-related prognostic model established in this study improves AML survival prediction accuracy.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"1-15"},"PeriodicalIF":1.7,"publicationDate":"2025-01-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142982325","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
Real-World Experience in the Management of Chronic Myeloid Leukemia Patients Focused on Tyrosine Kinase Inhibitors Intolerance and Health-Related Quality of Life. 在CML患者管理的实际经验集中在酪氨酸激酶抑制剂不耐受和健康相关的生活质量。
IF 1.7 4区 医学
Acta Haematologica Pub Date : 2025-01-10 DOI: 10.1159/000542562
Hee Jeong Cho, Dong Won Baek, Juhyung Kim, Young Eun Jang, Yunji Lee, Joon Ho Moon, Sang Kyun Sohn
{"title":"Real-World Experience in the Management of Chronic Myeloid Leukemia Patients Focused on Tyrosine Kinase Inhibitors Intolerance and Health-Related Quality of Life.","authors":"Hee Jeong Cho, Dong Won Baek, Juhyung Kim, Young Eun Jang, Yunji Lee, Joon Ho Moon, Sang Kyun Sohn","doi":"10.1159/000542562","DOIUrl":"10.1159/000542562","url":null,"abstract":"<p><strong>Introduction: </strong>This study aimed to analyze the survival outcomes and adverse events (AEs) associated with the long-term use of tyrosine kinase inhibitors (TKIs) and to assess health-related quality of life (HRQoL) in patients with chronic myeloid leukemia (CML).</p><p><strong>Methods: </strong>Medical records of 345 patients with CML treated with at least one type of TKI were retrospectively reviewed.</p><p><strong>Results: </strong>No significant differences in survival were observed based on the number of different TKIs the patients received (p = 0.301) or the sequence of TKIs used (p = 0.770). Among 182 patients treated with nilotinib, 25 experienced cardiovascular events (CVEs). After 10 years of nilotinib treatment, CVEs occurred in 55.2% of patients with ≥2 vascular risk factors. Pleural effusion was observed in 27 of 78 dasatinib-treated patients. In terms of HRQoL, patients treated with nilotinib generally reported higher satisfaction levels than did those treated with imatinib or dasatinib. When stratified by age or duration of TKI treatment, patients aged <60 years or those with a treatment duration of ≥1 year exhibited better satisfaction levels.</p><p><strong>Conclusion: </strong>Survival outcomes were not affected by history of TKI treatment. Nilotinib is favorable for HRQoL but increases the risk of serious CVEs in patients with vascular risk factors.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"1-14"},"PeriodicalIF":1.7,"publicationDate":"2025-01-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142969164","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
Direct STAT3 and STAT5 Inhibition Overcomes Treatment Resistance in a Murine-Derived in vitro Model of Acute Lymphoblastic Leukaemia Driven by ETV6::JAK2.
IF 1.7 4区 医学
Acta Haematologica Pub Date : 2025-01-06 DOI: 10.1159/000543428
Jane Frances Thompson, Randall Grose, David Yeung, Deborah L White
{"title":"Direct STAT3 and STAT5 Inhibition Overcomes Treatment Resistance in a Murine-Derived in vitro Model of Acute Lymphoblastic Leukaemia Driven by ETV6::JAK2.","authors":"Jane Frances Thompson, Randall Grose, David Yeung, Deborah L White","doi":"10.1159/000543428","DOIUrl":"https://doi.org/10.1159/000543428","url":null,"abstract":"<p><strong>Introduction: </strong>ETV6::JAK2 is a fusion known to drive acute lymphoblastic leukaemia (ALL) in the presence of other genomic lesions which define the JAK/STAT class of Philadelphia chromosome-like acute lymphoblastic leukaemia (Ph-like ALL). Ph-like ALL comprises approximately 15% of ALL. Patients with mutations or gene fusions signalling through the JAK/STAT pathway have particularly poor prognosis. Emerging treatments targeting JAK2 fusions and mutations are promising, and phase 3 clinical trials are in progress. However, with widespread use of JAK2 inhibitors, it is important to anticipate and manage resistance mechanisms. The JAK2 p.G993A mutation confers resistance in vitro, even to high-dose JAK2 inhibitors such as ruxolitinib. We postulated that direct inhibition of STAT3 and STAT5, downstream from JAK2, may overcome resistance.</p><p><strong>Methods: </strong>Murine-derived IL-3-dependent Ba/F3 cells were transfected with ETV6::JAK2 containing a p.G993A mutation for this study. These cells were confirmed to demonstrate IL-3 independence and ruxolitinib resistance prior to use in experiments. An inhibitor-response assay was conducted using differing concentrations of SH-4-54 and pimozide (STAT3/5 inhibitors) applied to ETV6::JAK2 p.G993A cells and two control cell lines.</p><p><strong>Result: </strong>SH-4-54 and pimozide were effective against ETV6::JAK2 p.G993A cells with median lethal doses (LD50) of 296 n<sc>M</sc> for SH-4-54 and 455 n<sc>M</sc> for pimozide. Both drugs demonstrated a lesser effect on empty vector Ba/F3 cells, with an LD50 of 371 n<sc>M</sc> for SH-4-54 and 596 n<sc>M</sc> for pimozide. Neither drug demonstrated significant effect on non-JAK/STAT-activated KG-1a myeloid cells at doses near the LD50.</p><p><strong>Conclusion: </strong>SH-4-54 and pimozide both overcame treatment resistance in our in vitro model of JAK/STAT-driven Ph-like ALL with a mutation conferring JAK2 inhibitor resistance. While SH-4-54 demonstrates greater potency than pimozide, pimozide may be a more promising option given its demonstrated safety profile in humans. Direct STAT3 and STAT5 inhibition may be an effective approach for overcoming inevitable JAK2 inhibitor resistance-conferring mutations in patients with the poor prognostic subtype of JAK/STAT class Ph-like ALL.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"1-5"},"PeriodicalIF":1.7,"publicationDate":"2025-01-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143381415","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
Initial Characterization and Outcome Assessment of Anal Lymphomas in a Large-Size Contemporary Cohort: A Population-Based SEER Database Study (2000-2022). 当代大规模队列中肛门淋巴瘤的初始特征和结果评估:一项基于人群的SEER数据库研究(2000-2022)。
IF 1.7 4区 医学
Acta Haematologica Pub Date : 2025-01-03 DOI: 10.1159/000541595
Pierre Loap, Youlia Kirova
{"title":"Initial Characterization and Outcome Assessment of Anal Lymphomas in a Large-Size Contemporary Cohort: A Population-Based SEER Database Study (2000-2022).","authors":"Pierre Loap, Youlia Kirova","doi":"10.1159/000541595","DOIUrl":"10.1159/000541595","url":null,"abstract":"<p><strong>Introduction: </strong>Anal lymphoma (AL) is a rare presentation of extranodal lymphomas, characterized by occurrence in the anal area and largely understudied due to its infrequency. This study aimed to address gaps in knowledge about AL's demographic and clinical profiles, treatments, and survival outcomes, leveraging data from the SEER program.</p><p><strong>Methods: </strong>We conducted a retrospective analysis of 79 AL cases identified in the SEER database from 2000 to 2022; 36 stage I AL cases were identified and defined as localized primary anal lymphoma (L-PAL). Data on demographics, tumor specifics, treatment modalities, and survival were analyzed using the Kaplan-Meier method and Cox proportional hazards models.</p><p><strong>Results: </strong>The majority of AL cases were diffuse large B-cell lymphoma (70.9%). Other notable subtypes included anaplastic T-cell lymphoma, marginal zone lymphoma, B-cell non-Hodgkin lymphoma, Burkitt lymphoma/leukemia (each accounting for 6.3%), followed by follicular lymphoma and mantle-cell lymphoma (each at 1.3%). AL primarily affected younger males (median age 50), with a significant majority being Caucasian. Initial stages (I and II) were more commonly observed, and treatments varied, with chemotherapy being most prevalent (67.1%), followed by radiation (30.4%) and surgery (30.4%). The 5- and 10-year overall survival (OS) rates were 59.4% and 44.1%, respectively, while the corresponding cancer-specific survival (CSS) rates were 67.9% and 58.0%, respectively. Age was a significant prognostic factor for OS but not for CSS. Radiotherapy tended to improve CSS in the AL population.</p><p><strong>Conclusion: </strong>This research corresponds to the first in-depth analysis of AL, highlighting its distinct demographic patterns, clinical features, and responses to various treatments, distinguishing it from other types of anal cancers. Our results underscore the importance of developing specialized diagnostic and treatment strategies. To enhance our understanding and management of this uncommon form of lymphoma, future studies should aim for broader and more collaborative international research efforts.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"1-7"},"PeriodicalIF":1.7,"publicationDate":"2025-01-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142930391","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
Evaluating the Economic Burden of Acute Myeloid Leukemia in Canada. 评估加拿大急性髓性白血病的经济负担。
IF 1.7 4区 医学
Acta Haematologica Pub Date : 2025-01-01 Epub Date: 2024-02-21 DOI: 10.1159/000537725
Jean Lachaine, Catherine Beauchemin, Fatéma Dodat, Yunghan Au, William K Evans, Brian Leber, Kristjan Paulson, Andre Schuh, John Storring
{"title":"Evaluating the Economic Burden of Acute Myeloid Leukemia in Canada.","authors":"Jean Lachaine, Catherine Beauchemin, Fatéma Dodat, Yunghan Au, William K Evans, Brian Leber, Kristjan Paulson, Andre Schuh, John Storring","doi":"10.1159/000537725","DOIUrl":"10.1159/000537725","url":null,"abstract":"<p><strong>Introduction: </strong>Acute myeloid leukemia (AML) represents a significant burden for patients and their families, and to the healthcare system. This study estimated the total cost of illness associated with newly diagnosed AML patients in Canada.</p><p><strong>Methods: </strong>The economic burden of AML was estimated using an incidence-based model, analyzing different types of AML cases in Canada. Direct and indirect costs were calculated using scientific literature and Canadian clinical experts' inputs. Patients were categorized depending on their eligibility for intensive chemotherapy (fit and unfit patients) as well as according to age and cytogenetic markers.</p><p><strong>Results: </strong>The total average cost of AML per patient is estimated to be CAD 178,073 with a cost of CAD 210,983 and CAD 145,163 for fit and unfit patients, respectively. The costs related to treatment represent half of the total average cost (52%), followed by hematopoietic stem cell transplant (23%), best supportive care (16%), productivity loss (6%), and wastage (4%).</p><p><strong>Conclusion: </strong>For patients with AML, the costs associated with fit patients are higher than unfit patients. Hospitalization and best supportive care costs are key cost drivers for the total costs of fit and unfit patients, respectively. This study highlights that AML is associated with a significant economic burden in Canada.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"8-21"},"PeriodicalIF":1.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139929471","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
HLA-DRB5 Overexpression Promotes Platelet Reduction in Immune Thrombocytopenia Mice Model by Facilitating MHC-II-Mediated Antigen Presentation. 通过促进 MHC-II 介导的抗原呈递,HLA-DRB5 的过表达可促进免疫性血小板减少症小鼠模型中血小板的减少。
IF 1.7 4区 医学
Acta Haematologica Pub Date : 2025-01-01 Epub Date: 2024-05-15 DOI: 10.1159/000538749
Yujuan Ren, Qianqian Ying, Ying Chen, Cong Liao, Anrong Li, Qidong Ye
{"title":"HLA-DRB5 Overexpression Promotes Platelet Reduction in Immune Thrombocytopenia Mice Model by Facilitating MHC-II-Mediated Antigen Presentation.","authors":"Yujuan Ren, Qianqian Ying, Ying Chen, Cong Liao, Anrong Li, Qidong Ye","doi":"10.1159/000538749","DOIUrl":"10.1159/000538749","url":null,"abstract":"<p><strong>Introduction: </strong>Major histocompatibility complex II (MHC-II)-mediated antigen presentation contributes to the pathogenesis of immune thrombocytopenia (ITP). Human leukocyte antigen (HLA)-DRB5 is an MHC-II molecule and this study aims to investigate its role and mechanisms in ITP development.</p><p><strong>Methods: </strong>Guinea pig anti-mouse platelet (PLT) serum-induced ITP mice received tail vein injection of HLA-DRB5 overexpressing adenoviral vector/immune receptor expressed on myeloid cells-1 (IREM-1) monoclonal antibody (mAb). PLT count changes in mice blood were assessed by a hematology analyzer. MHC-II/CD80/CD86 expression in mice blood was measured by quantitative real-time-PCR and immunofluorescence assay. CD8+ T-cell proportion in mice blood was detected by flow cytometry.</p><p><strong>Results: </strong>HLA-DRB5 overexpression exacerbated PLT reduction since the 5th day of the establishment of ITP mice model and enhanced MHC-II/CD80/CD86 expression upregulation as well as CD8+ T-cell ratio elevation in the blood of ITP mice, while its effects were reversed by IREM-1 mAb.</p><p><strong>Conclusion: </strong>HLA-DRB5 overexpression upregulates MHC-II-mediated antigen presentation to CD8+ T cells, thus lowering PLT count in the ITP mice model.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"68-76"},"PeriodicalIF":1.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140920761","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
Biologic and Clinical Characteristics of Isochromosome der(17)(q10)t(15;17) in Acute Promyelocytic Leukemia. 急性早幼粒细胞白血病中同源染色体 der(17)(q10)t(15;17) 的生物学和临床特征。
IF 1.7 4区 医学
Acta Haematologica Pub Date : 2025-01-01 Epub Date: 2024-05-31 DOI: 10.1159/000539159
Yuchen Liu, Yi Ning, Gabriel Ghiaur, Ashkan Emadi
{"title":"Biologic and Clinical Characteristics of Isochromosome der(17)(q10)t(15;17) in Acute Promyelocytic Leukemia.","authors":"Yuchen Liu, Yi Ning, Gabriel Ghiaur, Ashkan Emadi","doi":"10.1159/000539159","DOIUrl":"10.1159/000539159","url":null,"abstract":"<p><strong>Introduction: </strong>Acute promyelocytic leukemia (APL) is genetically characterized by the fusion of promyelocytic leukemia (PML) gene with retinoic acid receptor alpha (RARα) resulting from a t(15;17)(q24;q21) chromosomal translocation. An infrequent but recurrent finding in APL is the formation of an isochromosome of the derivative chromosome 17; ider(17)(q10)t(15;17) or ider(17q). This rearrangement in APL results in an additional copy of the PML-RARα fusion gene as well as loss of 17p/TP53. Due to the infrequent occurrence of the ider(17q), the prognostic impact of this genetic finding is not well known. Case Presentation(s): Here, we describe the clinical characteristics and outcomes of our case series of 5 patients with ider(17q) APL treated at the University of Maryland and Johns Hopkins University.</p><p><strong>Conclusion: </strong>In our series, patients with APL with ider(17q) did not have a worse prognosis.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"111-118"},"PeriodicalIF":1.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141199230","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
Factor VIII Levels and ISTH Disseminated Intravascular Coagulation Scores Do Not Distinguish Disseminated Intravascular Coagulation from the Coagulopathy of Liver Disease. 因子 VIII 水平和 ISTH DIC 评分并不能区分 DIC 和肝病凝血病。
IF 1.7 4区 医学
Acta Haematologica Pub Date : 2025-01-01 Epub Date: 2024-07-16 DOI: 10.1159/000540239
Cecily Allen, Marina Heskel, Ayesha Butt, Christopher Tormey, Alexander B Pine, Alfred I Lee, Samir Gautam
{"title":"Factor VIII Levels and ISTH Disseminated Intravascular Coagulation Scores Do Not Distinguish Disseminated Intravascular Coagulation from the Coagulopathy of Liver Disease.","authors":"Cecily Allen, Marina Heskel, Ayesha Butt, Christopher Tormey, Alexander B Pine, Alfred I Lee, Samir Gautam","doi":"10.1159/000540239","DOIUrl":"10.1159/000540239","url":null,"abstract":"<p><strong>Introduction: </strong>Distinguishing disseminated intravascular coagulation (DIC) from the coagulopathy of liver disease represents a common clinical challenge. Here, we evaluated the utility of two diagnostic tools frequently used to differentiate between these conditions: factor VIII (FVIII) levels and the International Society on Thrombosis and Hemostasis (ISTH) DIC score.</p><p><strong>Methods: </strong>To this end, we conducted a retrospective chart review of patients with DIC, liver disease, or both. Multiple logistic regression was performed, and receiver operating characteristic curves were generated to calculate the area under curve (AUC) for distinguishing DIC in the setting of liver disease.</p><p><strong>Results: </strong>Among 123 patients with DIC, liver disease, or liver disease plus DIC, FVIII levels did not differ significantly. ISTH scores were lower in patients with DIC than in liver disease with or without DIC. Addition of several laboratory parameters to the ISTH score, including mean platelet volume, FV, FVIII, international normalized ratio, and activated partial thromboplastin time, improved AUC for distinguishing DIC in liver disease from liver disease alone (AUC = 0.76; p < 0.0001).</p><p><strong>Conclusion: </strong>We conclude that FVIII levels do not distinguish DIC from liver disease, and ISTH DIC scores are not predictive of DIC in patients with liver disease. Inclusion of additional lab variables within the ISTH DIC score may aid in identifying DIC in patients with liver disease.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"220-225"},"PeriodicalIF":1.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141615632","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
Retrospective Evaluation of Oral Glucose Tolerance Test in Young Patients with Transfusion-Dependent Beta-Thalassemia. 口服葡萄糖耐量试验(OGTT)对年轻输血依赖性β地中海贫血患者的回顾性评价。
IF 1.7 4区 医学
Acta Haematologica Pub Date : 2025-01-01 Epub Date: 2022-03-02 DOI: 10.1159/000523874
Maria Dritsa, Marina Economou, Vasilios Perifanis, Aikaterini Teli, Athanasios Christoforidis
{"title":"Retrospective Evaluation of Oral Glucose Tolerance Test in Young Patients with Transfusion-Dependent Beta-Thalassemia.","authors":"Maria Dritsa, Marina Economou, Vasilios Perifanis, Aikaterini Teli, Athanasios Christoforidis","doi":"10.1159/000523874","DOIUrl":"10.1159/000523874","url":null,"abstract":"<p><strong>Background: </strong>Current guidelines recommend yearly evaluation of glucose metabolism with oral glucose tolerance test (OGTT) in patients with thalassemia major (TM) from their 10th year onwards.</p><p><strong>Aims: </strong>The aim of the study was to retrospectively evaluate all the OGTT tests performed in a single pediatric center during the last decade and assess the necessity of yearly performed OGTT in patients with TM during the second decade of life.</p><p><strong>Results: </strong>One hundred and seventy tests performed on 39 patients (24 boys) were included in the analysis. Mean age at the time of each OGTT was 14.15 ± 2.79 years. Seventeen tests (10%) in 8 patients (5 boys) were characterized as impaired glucose tolerance (IGT), whereas in 2 tests (1.18%), fasting glucose values were above 200 mg/dL (diabetes mellitus). Acknowledging the fact that there could be sporadic technical errors, especially in fasting glucose abnormal values, we selectively separated patients with either IGT and abnormal HOMA, MATSUDA, or QUICKI indices or persistently impaired fasting glucose or IGT. With these criteria, 7 patients (4 boys) were identified. No significant differences were observed in demographic and anthropometric parameters, ferritin levels, and mean volume of blood transfused in patients with a definite abnormal glucose metabolism. In order to address selection criteria for performing OGTT without missing a single patient with a definite glucose intolerance, these criteria should include (i) a first OGTT test at the initiation of puberty, (ii) OGTT in any patient with a fasting glucose above 100 mg/dL, and (iii) OGTT in any patient with a ΗΟΜΑ-IR index above 1.85 and yearly onwards. By adopting these criteria, total OGTT tests performed could have been reduced from 170 to only 91, translating to a significant reduction of 46.47%.</p><p><strong>Conclusions: </strong>Only a small percentage of young patients with transfusion-dependent b-thalassemia exhibit abnormal glucose metabolism during their second decade of life. By adopting specific selection criteria, one may avoid performing this time- and money-consuming test in a significant proportion of patients, albeit without sacrificing timely detection and intervention.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":"1 1","pages":"1-7"},"PeriodicalIF":1.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43094096","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}
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