Advances in HematologyPub Date : 2025-05-19eCollection Date: 2025-01-01DOI: 10.1155/ah/2179600
Afshan Sumera, Ammu K Radhakrishnan, Soon Keng Cheong, Abdul Aziz Baba
{"title":"Dysregulation of Ceruloplasmin, α2-Macroglobulin, and Alpha-2-HS-Glycoprotein in Transfusion-Dependent Thalassemia.","authors":"Afshan Sumera, Ammu K Radhakrishnan, Soon Keng Cheong, Abdul Aziz Baba","doi":"10.1155/ah/2179600","DOIUrl":"https://doi.org/10.1155/ah/2179600","url":null,"abstract":"<p><p>Transfusion-dependent thalassemia (TDT) is a severe inherited anemia characterized by impaired synthesis of hemoglobin chains. Disease progression and TDT severity are potentially linked to oxidative stress and protein damage. This study aimed to explore the expression patterns of ceruloplasmin (CP), α2-macroglobulin (A2M), and alpha-2-HS-glycoprotein (AHSG) in TDT serum through quantitative proteomic profiling. The results were validated using enzyme-linked immunosorbent assays (ELISA). The study participants were divided into three groups based on the duration of blood transfusion. Age and gender-matched normal individuals served as controls. The results revealed the downregulation of these proteins. The reduced levels of these proteins may contribute to tissue damage in TDT patients, primarily due to increased oxidative stress. For example, decreased CP levels can disrupt iron and copper metabolism, leading to heightened oxidative stress and rendering red blood cell membranes more susceptible to rupture due to active oxygen radicals. In summary, CP, A2M, and AHSG association with iron metabolism, inflammation, and oxidative stress underscores their potential relevance in understanding TDT's pathogenesis and progression. These findings may pave the way for improved diagnostic and therapeutic strategies for TDT patients.</p>","PeriodicalId":7325,"journal":{"name":"Advances in Hematology","volume":"2025 ","pages":"2179600"},"PeriodicalIF":0.0,"publicationDate":"2025-05-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12105889/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144148810","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}
Advances in HematologyPub Date : 2025-05-06eCollection Date: 2025-01-01DOI: 10.1155/ah/9971532
Catherine Segbefia, Susan Telke, Edeghonghon Olayemi, Caitlin Ward, Lucy Asamoah-Akuoko, Bernard Appiah, Alfred Edwin Yawson, Tara Tancred, Seth Adu-Afarwuah, Amma Benneh-Akwasi Kuma, Michael Ebo Acquah, Solomon Fiifi Ofori-Acquah, Philip Baba Adongo, Reena Ametorwo, Imelda Bates, Cavan Reilly, Yvonne Dei-Adomakoh
{"title":"Deferrals for Low Haemoglobin and Anaemia Among First-Time Prospective Blood Donors in Southern Ghana: Results From the BLOODSAFE Ghana-Iron and Nutritional Counselling Strategy Pilot (BLIS) Study.","authors":"Catherine Segbefia, Susan Telke, Edeghonghon Olayemi, Caitlin Ward, Lucy Asamoah-Akuoko, Bernard Appiah, Alfred Edwin Yawson, Tara Tancred, Seth Adu-Afarwuah, Amma Benneh-Akwasi Kuma, Michael Ebo Acquah, Solomon Fiifi Ofori-Acquah, Philip Baba Adongo, Reena Ametorwo, Imelda Bates, Cavan Reilly, Yvonne Dei-Adomakoh","doi":"10.1155/ah/9971532","DOIUrl":"10.1155/ah/9971532","url":null,"abstract":"<p><p>In Ghana, prevalence of anaemia is higher than the worldwide average and contributes to deferral of blood donors. A cross-sectional study was carried out as part of a pilot study aimed at improving haemoglobin levels and promoting repeat donations to retain donors who were deferred due to low haemoglobin. The copper sulphate test was used to determine low haemoglobin and anaemia assessed by the World Health Organization (WHO) gender-specific criteria. Over sixteen months, 1213 donors were eligible, of which 826 (68%) were male and 78 (6.4%) were deferred for low haemoglobin. Among these 78 deferrals, 71 (91%) were female, 77 (99%) were first-time donors and 77 (99%) were voluntary nonremunerated blood donors (VNRBDs). A total of 337 donors consented to provide a blood specimen out of which 325 donors met eligibility criteria and had complete FBC results. Of those, 189 (<i>N</i> = 39 males; <i>N</i> = 150 females), or 58%, were classified as anaemic. Model-based estimates which correct for selection bias in the enrolment process found that 61.6% of female donors (95% credible interval: [53.4%, 70.8%]) and 19.7% of male donors (95% credible interval: [11.5%, 33.8%]) were anaemic by WHO criteria. Among the 252 consenting donors with completed blood specimen analyses and haemoglobin levels meeting the threshold for blood donation, 118 (47%) were classified as anaemic according to WHO criteria. Population-level estimates of anaemia using WHO criteria suggest anaemia is highly prevalent and the results generally matched donor deferral using the copper sulphate test among women blood donors. <b>Trial Registration:</b> ClinicalTrials.gov identifier: NCT04949165.</p>","PeriodicalId":7325,"journal":{"name":"Advances in Hematology","volume":"2025 ","pages":"9971532"},"PeriodicalIF":0.0,"publicationDate":"2025-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12074844/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143959273","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}
Advances in HematologyPub Date : 2025-04-21eCollection Date: 2025-01-01DOI: 10.1155/ah/8080147
Tandry Meriyanti, Maroloan Aruan, Glorya N D Ananda
{"title":"Role of Extended White Blood Cell Parameters in Distinguishing Acute Febrile Illnesses.","authors":"Tandry Meriyanti, Maroloan Aruan, Glorya N D Ananda","doi":"10.1155/ah/8080147","DOIUrl":"https://doi.org/10.1155/ah/8080147","url":null,"abstract":"<p><p><b>Introduction:</b> Acute febrile illness contributes to significant morbidity and death particularly in tropical country such as Indonesia. The symptoms are nonspecific, therefore distinguishing these pathogens is difficult without additional laboratory tests. The extended white blood cell parameters indicate cell activities induced by immune response to infection. The study aims to explore the profile of extended white blood cell parameters in acute febrile illnesses and evaluate their diagnostic power to differentiate etiologies of acute febrile illnesses. <b>Methods:</b> This study was a cross-sectional analytical study with a total of 473 samples, conducted between October 2022 and 2023 at Siloam Hospitals Lippo Village, Banten, Indonesia. Acute febrile illnesses are included in this study, including dengue infection, chikungunya infection, typhoid infection, and other bacterial infections. The extended white blood cell parameters including high fluorescence lymphocyte count (HFLC), immature granulocyte (IG), neutrophil-to-lymphocyte ratio (NLR), and cell population data (CPD) which were NE-SSC, NE-SFL, NE-WY, LY-X, LY-Y, and LY-WY. These parameters were integrated in a routine hematology test as research parameters, performed by Sysmex XN2000. Data were analyzed using SPSS Version 25. <b>Results:</b> The value of extended white blood cell parameters was found to be significantly different in viral and bacterial infection (HFLC 1.10% (0.30%-3.85%) vs. 0.20% (0.10%-0.70%), <i>p</i> < 0.001; IG 0.4% (0.2%-0.6%) vs. 0.5% (0.3%-1.1%), <i>p</i> < 0.001; NLR 1.93 (1.10-3.47) vs. 5.21 (2.20-12.26), <i>p</i> < 0.001; NE-SFL 47.7 (45.95-50.10) vs. 48.6 (45.82-52.57), <i>p</i>=0.020; NE-WY 622 (585-653) vs. 653 (615-747), <i>p</i> < 0.001; LY-Y 66.4 (63.85-69.75) vs. 64.05 (60.52-67.17), <i>p</i> < 0.001). HFLC and LY-Y had statistically significant AUC 0.753 and 0.646, respectively, (<i>p</i> < 0.001) in the dengue infection group. IG, NLR, NE-WY, and NE-SFL had statistically significant AUC in bacteremia (0.806, 0.876, 0.783, and 0.656, respectively). <b>Conclusion:</b> HFLC was a useful diagnostic tool to identify viral infection, particularly dengue infection, while IG, NLR, NE-SFL, and NE-WY can be useful to differentiate bacteremia from other acute febrile illnesses.</p>","PeriodicalId":7325,"journal":{"name":"Advances in Hematology","volume":"2025 ","pages":"8080147"},"PeriodicalIF":0.0,"publicationDate":"2025-04-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12037241/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143954912","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}
Advances in HematologyPub Date : 2025-03-19eCollection Date: 2025-01-01DOI: 10.1155/ah/8871102
Jean Baptiste Niyibizi, Daniel Seifu, Chelsey Geurkink, Erica Formiller, Thomas Muyombo, Christopher Gashaija, Henri Desire Uwayo, Gilbert Uwizeyimana, Laurie Gillard
{"title":"Assessment of Unexpected (Non-ABO) Red Blood Cell Antibodies and Their Associated Clinical Conditions Among Patients and Blood Donors Attending University Teaching Hospital of Kigali (CHUK) and Rwanda Blood Transfusion Division.","authors":"Jean Baptiste Niyibizi, Daniel Seifu, Chelsey Geurkink, Erica Formiller, Thomas Muyombo, Christopher Gashaija, Henri Desire Uwayo, Gilbert Uwizeyimana, Laurie Gillard","doi":"10.1155/ah/8871102","DOIUrl":"10.1155/ah/8871102","url":null,"abstract":"<p><p>Unexpected antibodies can cause hemolytic conditions. Therefore, screening for unexpected antibodies is essential for safe transfusion. The study was conducted at Rwanda Blood Transfusion Division and University Teaching Hospital of Kigali to assess unexpected antibodies with their associated clinical conditions. 8693 blood donors and 834 patients were screened for unexpected antibodies. Among 834 patients, 23 patients (2.75%) developed alloantibodies among which two of them had mixed alloantibodies. Five patients developed antibodies of uncertain specificities. Among 8693 blood donors, only 4 blood donors (0.046%) had clinically significant alloantibodies, whereas 6 blood donors (0.069%) had antibodies of uncertain specificities. Moreover, 3 patients (0.35%) had autoantibodies in their plasma. Different types of anemia were presented with patients who developed unexpected alloantibodies. History of transfusion and pregnancy were predictors of alloimmunization among patients (<i>p</i> < 0.01). Antibody screening and antibody identification are important for safe blood transfusion practices.</p>","PeriodicalId":7325,"journal":{"name":"Advances in Hematology","volume":"2025 ","pages":"8871102"},"PeriodicalIF":0.0,"publicationDate":"2025-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11944671/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143727308","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}
Advances in HematologyPub Date : 2025-03-03eCollection Date: 2025-01-01DOI: 10.1155/ah/7280805
Margaret Locke, Maria Nieto
{"title":"AL Amyloidosis: Current Treatment and Outcomes.","authors":"Margaret Locke, Maria Nieto","doi":"10.1155/ah/7280805","DOIUrl":"https://doi.org/10.1155/ah/7280805","url":null,"abstract":"<p><p>Light chain AL amyloidosis is a systemic disorder involving tissue deposition of amyloid fibrils. Often delayed in diagnosis due to nonspecific systemic symptoms, AL amyloidosis must be confirmed on tissue biopsy. Once diagnosis is made, patients can be risk stratified based on the degree of organ involvement and high-risk cytogenetic features. Currently, the only FDA-approved first-line therapy for AL amyloidosis is a combination regimen of daratumumab, cyclophosphamide, bortezomib, and dexamethasone (DaraCyborD) with a goal of achieving a very good partial response (VGPR) after 4-6 cycles of treatment. Autologous stem cell transplant can be considered in selected cases, although there is no robust evidence of superiority over chemotherapy alone. In the relapsed/refractory setting, numerous promising therapies are still under investigation including venetoclax especially for patients with translocation t (11; 14) and chimeric antigen receptor T-cell therapy (CART) targeting B-cell maturation antigen (BCMA). <b>Trial Registration:</b> ClinicalTrials.gov identifier: NCT04270175, NCT05451771, NCT04847453, and NCT05199337.</p>","PeriodicalId":7325,"journal":{"name":"Advances in Hematology","volume":"2025 ","pages":"7280805"},"PeriodicalIF":0.0,"publicationDate":"2025-03-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11991823/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143952144","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}
Advances in HematologyPub Date : 2024-12-19eCollection Date: 2024-01-01DOI: 10.1155/ah/3854629
Jayla Lynn Scott, Jana Christian, Manuela Plazas Montana, Yvette M Miller, Rakhi P Naik
{"title":"Variability in Notification of Positive Newborn Screening Results for Sickle Cell Trait Across the United States.","authors":"Jayla Lynn Scott, Jana Christian, Manuela Plazas Montana, Yvette M Miller, Rakhi P Naik","doi":"10.1155/ah/3854629","DOIUrl":"10.1155/ah/3854629","url":null,"abstract":"<p><p>Universal in the United States (US) since 2006, newborn screening (NBS) programs for sickle cell disease (SCD) allow for early identification of the disease and, as an unintentional byproduct, identification of sickle cell trait (SCT). Unlike other carrier states, SCT is highly prevalent and is found in nearly 3 million Americans, which results in important reproductive implications. Currently, all NBS programs in the US are responsible for their own policies regarding SCT notification, and little is known about how SCT notification practices are performed and how these practices vary across NBS programs. We surveyed NBS programs personnel in all 50 states, the District of Columbia, and the US' territories of Puerto Rico and Guam (<i>n</i> = 53) using an electronic survey. There was a 100% response rate. All NBS programs (100%) provide notification of SCT status to either a pediatrician or parent: 49% notify the pediatrician only, 45% notify both the pediatrician and parent, and 6% notify the parent only. A total of 98% of NBS programs retain electronic records of SCT status, but only 38% can be directly accessed by pediatricians/primary care doctors. No state operates a publicly available database that allows individuals to access their own records. Only one state provides renotification at reproductive age. In conclusion, there is wide variability in NBS practices for SCT notification. This study demonstrates a need for national guidelines to standardize SCT notification across the US to ensure effective notification and counseling for SCT.</p>","PeriodicalId":7325,"journal":{"name":"Advances in Hematology","volume":"2024 ","pages":"3854629"},"PeriodicalIF":0.0,"publicationDate":"2024-12-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11671650/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142902315","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}
{"title":"Early Molecular Response to Imatinib First-Line Therapy and Predictive Factors of Poor Outcomes for Chronic Myeloid Leukemia Patients in Côte d'Ivoire.","authors":"Kouassi Gustave Koffi, Sara Akou Bognini, Dohoma Alexis Silué, Ismael Kamara, Ines Kouakou, Emeraude N'dhatz, Boidy Kouakou, Danho Clotaire Nanho, David Tea Okou","doi":"10.1155/2024/4576455","DOIUrl":"10.1155/2024/4576455","url":null,"abstract":"<p><p><b>Objective:</b> The present study aimed to evaluate for the first time, the early molecular response (EMR) to imatinib at 3 months for patients with chronic myeloid leukemia and to determine the predictive factors that influence poor outcome and response. <b>Methods:</b> 60 newly diagnosed CML patients were enrolled from May 2018 to June 2023. They received imatinib and prospectively underwent a molecular evaluation. Their EMR was assessed using a RT-qPCR method and expressed as the <i>BCR::ABL1</i> IS transcript level at 3 months. Potential factors impacting the EMR were identified using the Cox proportional hazard regression models. The effects of an EMR on the cumulative incidence of a deep molecular response (DMR) were also evaluated. <b>Results:</b> Out of the 60 CML patients recruited, 29 (48%) achieved an optimal response with TKI therapy after 3 months. The cumulative rate of molecular response was 16 (36%) for a major molecular response (MMR), 10 (23%) for MR4, 8 (18%) for MR4.5, and 6 (14%) for MR5, while 4 (9%) showed indetectable transcript. In addition, as 26 (90%) of patients with optimal response at 3 months showed a DMR, we determined that an optimal response to TKI at 3 months was significantly correlated with a DMR. We also identified through multivariate analysis that seven independent risk factors significantly influenced an EMR to TKI. These factors included male, late diagnosis, advanced performance status, the presence of splenomegaly, high-ELTS risk groups, a <i>BCR::ABL1</i> domain mutation, and complete hematologic response after more than 30 days. <b>Conclusion:</b> Our study demonstrates that an EMR at 3 months has a predictive value for a DMR. In addition, a MMR and a DMR can be predicted using a combination of parameters that either have a significant impact on the optimal response, or that can serve as prognostic indicators for molecular response, especially in low-income countries, where molecular assessment and monitoring are not available or possible.</p>","PeriodicalId":7325,"journal":{"name":"Advances in Hematology","volume":"2024 ","pages":"4576455"},"PeriodicalIF":0.0,"publicationDate":"2024-12-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11655142/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142851592","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}
Advances in HematologyPub Date : 2024-12-03eCollection Date: 2024-01-01DOI: 10.1155/ah/9872440
Nermi L Parrow, Jason M Doherty, Anna Conrey, Swee Lay Thein, Robert E Fleming
{"title":"Relationships Between Markers of Iron Status and Hematological Parameters in Patients With Sickle Cell Disease.","authors":"Nermi L Parrow, Jason M Doherty, Anna Conrey, Swee Lay Thein, Robert E Fleming","doi":"10.1155/ah/9872440","DOIUrl":"10.1155/ah/9872440","url":null,"abstract":"<p><p>Based on the relationship between the intracellular concentration of sickle hemoglobin S (HbS) and the delay that occurs prior to the onset of sickling following deoxygenation, targeting the intracellular HbS concentration is a recognized therapeutic approach for sickle cell disease (SCD). We and others have shown that restricting iron by dietary or pharmacologic means improves hematologic parameters, inflammation, and organ damage in mouse models of SCD. Clinical evidence corroborating these findings is confined to case reports and small case series studies, none of which account for treatment or <i>α</i>-thalassemia. We hypothesize that increased transferrin saturation is associated with increased mean cellular hemoglobin concentration (MCHC) which in turn is associated with decreased red cell counts and worsening anemia. To investigate this hypothesis, we examined the relationships between transferrin saturation and MCHC with each of the parameters that define MCHC in sickle patients (HbSS without <i>α</i>-thalassemia) and healthy volunteers (HVs). Results indicate that transferrin saturation and MCHC are positively correlated with each other in sickle patients and HV. In patients with SCD, MCHC and transferrin saturation are negatively correlated with RBC count and are not correlated with hemoglobin, whereas each is positively associated with HV. Transferrin saturation and MCHC are each positively correlated with the hemolysis marker, lactate dehydrogenase. These observations support a model where increased transferrin saturation contributes to higher intracellular HbS concentrations with subsequent increases in sickling and hemolysis in sickle patients, suggesting that pharmacologic approaches to decrease serum iron may provide a therapeutic approach for patients with SCD. <b>Trial Registration:</b> This study was registered with ClinicalTrials.gov identifiers: NCT00011648, NCT00081523, and NCT04817670.</p>","PeriodicalId":7325,"journal":{"name":"Advances in Hematology","volume":"2024 ","pages":"9872440"},"PeriodicalIF":0.0,"publicationDate":"2024-12-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11631288/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142805969","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}
Advances in HematologyPub Date : 2024-11-23eCollection Date: 2024-01-01DOI: 10.1155/ah/5002373
Blessing E Kene-Udemezue, Abideen O Salako, Adeseye M Akinsete, Oluwatosin O Odubela, Titilope A Adeyemo
{"title":"A Comparative Analysis of Hydroxyurea Treatment on Coagulation Profile Among Sickle Cell Anaemia Children in Lagos, Nigeria.","authors":"Blessing E Kene-Udemezue, Abideen O Salako, Adeseye M Akinsete, Oluwatosin O Odubela, Titilope A Adeyemo","doi":"10.1155/ah/5002373","DOIUrl":"https://doi.org/10.1155/ah/5002373","url":null,"abstract":"<p><p><b>Background:</b> Hydroxyurea (HU) is a disease-modifying therapy with significant clinical and laboratory efficacy among individuals living with sickle cell anaemia (SCA). This is evident through increased fetal haemoglobin, higher packed cell volume, improved red cell hydration, reduced leukocytes, and platelet function. The effect on the coagulation pathway and pathophysiologic mechanism remains unclear, especially in children living with SCA. This study evaluated the coagulation profile using D-dimer and thrombin antithrombin complex (TAT) in children with SCA. <b>Methods:</b> The cross-sectional study was conducted over three months at LUTH among 80 children living with SCA in steady state aged 2-18 years (40 HU exposed and 40 HU naïve, respectively). Blood samples were assayed for D-dimer, TAT, and complete blood count. Descriptive analysis such as mean and standard deviation for normally distributed variables or median and interquartile range for skewed data were used to summarize continuous variables, while proportion or percentages for categorical variables. Univariate analysis and bivariate analysis were done and statistical significance was set at <i>p</i> < 0.05. <b>Results:</b> The mean age (±SD) of study participants in both groups was 11.35 (±4.6 years). D-dimer levels (23.27 ng/mL) and TAT (29.79 pg/mL) were significantly lower among HU exposed compared to HU naïve children (62.73 ng/mL and 109.34 pg/mL, respectively) <i>p</i> < 0.001. There was a negative correlation between D-dimer and TAT with the duration of HU use (<i>r</i> = -0.499, <i>p</i>=0.001, and <i>r</i> = -0.401, <i>p</i>=0.010), respectively. There was a positive correlation between D-dimer and TAT with total WBC (<i>r</i> = 0.368, <i>p</i>=0.019, and <i>r</i> = 0.385, <i>p</i>=0.014, respectively) among the HU naïve participants and a negative correlation between D-dimer and TAT with haemoglobin level (<i>r</i> = -0.303, <i>p</i>=0.047, and <i>r</i> = -0.311, <i>p</i>=0.041, respectively) among HU exposed children. <b>Conclusion:</b> HU modulates the D-dimer and TAT levels of children living with SCA toward the normal reference range, thus reducing the risk of hypercoagulability and associated sequelae. Therefore, continuous advocacy for HU use should entail close monitoring of adverse effects.</p>","PeriodicalId":7325,"journal":{"name":"Advances in Hematology","volume":"2024 ","pages":"5002373"},"PeriodicalIF":0.0,"publicationDate":"2024-11-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11611399/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142765441","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}
Advances in HematologyPub Date : 2024-11-12eCollection Date: 2024-01-01DOI: 10.1155/2024/5948170
Abdullah Alfaifi, Salem Bahashwan, Mohammed Alsaadi, Ali H Ageel, Hamzah H Ahmed, Kaneez Fatima, Hafiz Malhan, Ishtiaq Qadri, Hussein Almehdar
{"title":"Advancements in B-Cell Non-Hodgkin's Lymphoma: From Signaling Pathways to Targeted Therapies.","authors":"Abdullah Alfaifi, Salem Bahashwan, Mohammed Alsaadi, Ali H Ageel, Hamzah H Ahmed, Kaneez Fatima, Hafiz Malhan, Ishtiaq Qadri, Hussein Almehdar","doi":"10.1155/2024/5948170","DOIUrl":"10.1155/2024/5948170","url":null,"abstract":"<p><p>Lymphoma is the sixth most prevalent cancer globally. Non-Hodgkin's lymphomas are the majority group of lymphomas, with B cells accounting for approximately 95% of these lymphomas. A key feature of B-cell lymphoma is the functional perturbations of essential biological pathways caused by genetic aberrations. These lead to atypical gene expression, providing cells with a selective growth advantage. Molecular analysis reveals that each lymphoma subtype has unique molecular mutations, which pose challenges in disease management and treatment. Substantial efforts over the last decade have led to the integration of this information into clinical applications, resulting in crucial insights into clinical diagnosis and targeted therapies. However, with the growing need for more effective medication development, we anticipate a deeper understanding of signaling pathways and their interactions to emerge. This review aims to demonstrate how the BCR, specific signaling pathways like PI3K/AKT/mTOR, NF-kB, and JAK/STAT are diverse in common types of B-cell lymphoma. Furthermore, it offers a detailed examination of each pathway and a synopsis of the approved or in-development targeted therapies. In conclusion, finding the activated signaling pathways is crucial for developing effective treatment plans to improve the prognosis of patients with relapsed or refractory lymphoma. <b>Trial Registration:</b> ClinicalTrials.gov identifier: NCT02180724, NCT02029443, NCT02477696, NCT03836261, NCT02343120, NCT04440059, NCT01882803, NCT01258998, NCT01742988, NCT02055820, NCT02285062, NCT01855750, NCT03422679, NCT01897571.</p>","PeriodicalId":7325,"journal":{"name":"Advances in Hematology","volume":"2024 ","pages":"5948170"},"PeriodicalIF":0.0,"publicationDate":"2024-11-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11576080/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142674886","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}