{"title":"Transition Readiness of Pediatric Sickle Cell Patients to Adult Clinic in a Teaching Hospital, Ghana.","authors":"Aaron Kwasi Nartey, Vivian Paintsil, Isaac Nyanor, Yaa Gyamfua Oppong-Mensah, Evans Xorse Amuzu, Eunice Agyeman Ahmed, Suraj Yawnumah Abubakar, Alex Osei-Akoto","doi":"10.1155/ah/2843974","DOIUrl":"https://doi.org/10.1155/ah/2843974","url":null,"abstract":"<p><p><b>Background:</b> Successfully navigating the transition process has received little attention, especially in sub-Saharan Africa. This study assessed the transition readiness of pediatric sickle cell disease (SCD) patients in the Komfo Anokye Teaching Hospital (KATH), Kumasi-Ghana. <b>Methods:</b> A hospital-based cross-sectional study was conducted using a purposive sampling technique to recruit adolescents who were scheduled to be transitioned from the Pediatric to the Adult SCD Clinic at KATH. Two transition assessment tools were adopted and modified to suit our local setting. <b>Findings:</b> Majority of the patients (90%) scored above median mark for the items under the transition self-care importance and confidence and over 50% for most of the items under the disease knowledge and appointment keeping domains. The internal consistencies of the items were over 70% for all the three domains: disease knowledge, medication management, and appointment keeping. In multivariable regression models, older age, female gender, and higher education were associated with higher scores in all the three domains. Also, the sickle cell disease-SS (SCD-SS) status was associated with higher scores in disease knowledge and appointment keeping. Patients staying with both parents were associated with higher scores for the domains but only appointment keeping was statistically significant. Staying with other relations was associated with a lower score for appointment keeping and had significant association for medication management. <b>Conclusion:</b> The study revealed a high transition readiness among pediatric patients. In general, the patients had high confidence transitioning to an adult clinic and the ability to manage their own healthcare. However, patients were hesitant speaking about their SCD status. Staying with both parents was significantly associated with higher scores for appointment keeping. Also, staying with other relations significantly reduced the scores for medication management. We recommend setting up of an adolescent sickle cell support group to help reduce stigmatization and improve health outcomes.</p>","PeriodicalId":7325,"journal":{"name":"Advances in Hematology","volume":"2025 ","pages":"2843974"},"PeriodicalIF":0.0,"publicationDate":"2025-07-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12271718/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144673702","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 : 2025-06-28eCollection Date: 2025-01-01DOI: 10.1155/ah/6934791
Collince O Ogolla, Rodgers N Demba
{"title":"Evaluation of Bacterial Contamination in Donated Blood for Transfusion Purposes at Kisii Teaching and Referral Hospital.","authors":"Collince O Ogolla, Rodgers N Demba","doi":"10.1155/ah/6934791","DOIUrl":"10.1155/ah/6934791","url":null,"abstract":"<p><p><b>Background:</b> Bacterial contamination of donated blood has been a major public health problem. It poses grave risks to the recipient. <b>Objective:</b> The objective of this study was to determine bacterial contamination in donated blood for transfusion purposes at Kisii Teaching and Referral Hospital. <b>Methodology:</b> This was a cross-sectional study. Sample collection was performed in BD BACTEC culture bottles and analyzed by BD BACTEC Machine FX40 for the presence of bacteria and thereafter subcultured for the positive vials. Biochemical tests were performed followed by confirmation tests with API-20 to identify bacterial presence. Samples negative for bacteria were not subjected to further analysis, and the results were directly recorded. Quality control procedures were performed using known ATCC microorganisms (<i>Staphylococcus aureus</i> [<i>S. aureus</i>] ATCC 25923). The data were entered into Excel and analyzed by SPSS Version 25. <b>Results:</b> The general prevalence of bacterial contamination was 21.3% (23/108). The blood group A positive had the highest contamination rate (10.2%), while the prevalence by age was also higher in the 21-30 years age group (24%). The most commonly isolated organisms were <i>Staphylococcus epidermidis</i> (<i>S. epidermidis</i>) (56.5%), <i>S. aureus</i> (39.1%), <i>Bacillus</i> spp. (30.4%), and <i>Escherichia coli</i> (<i>E. coli</i>) (17.4%). Logistic regression analysis indicated that blood group A positive individuals (OR = 2.5, <i>p</i>=0.02) and the 21-30 years age group (OR = 1.8, <i>p</i>=0.03) were significantly related to contamination at odds. The association of blood group A positive and age 21-30 further augmented this risk (OR = 3.5, <i>p</i>=0.01). <b>Conclusion:</b> <i>S. epidermidis</i>, <i>S. aureus</i>, <i>Bacillus</i> spp. and <i>E. coli</i> were among the isolated and identified bacteria found in donated blood samples among donors at KTRH.</p>","PeriodicalId":7325,"journal":{"name":"Advances in Hematology","volume":"2025 ","pages":"6934791"},"PeriodicalIF":0.0,"publicationDate":"2025-06-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12256170/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144635961","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 : 2025-06-17eCollection Date: 2025-01-01DOI: 10.1155/ah/5513287
Andrew Picca, David Kling, Amanda Jacobson-Kelly, Kathleen Nicol, Joseph Stanek, Vilmarie Rodriguez
{"title":"Reduction of Blood Transfusion in Iron Deficiency Anemia (ReBIDA): A Quality Improvement Initiative.","authors":"Andrew Picca, David Kling, Amanda Jacobson-Kelly, Kathleen Nicol, Joseph Stanek, Vilmarie Rodriguez","doi":"10.1155/ah/5513287","DOIUrl":"10.1155/ah/5513287","url":null,"abstract":"<p><p><b>Background:</b> Iron deficiency anemia (IDA) is the most common form of pediatric anemia, with first-line treatment focusing on iron repletion through oral and/or intravenous iron. The American Society of Hematology (ASH)/the American Society of Pediatric Hematology and Oncology (ASPHO) Choosing Wisely Campaign recommends against packed red blood cell (PRBC) transfusion for asymptomatic IDA. PRBCs are a finite resource and carry treatment associated risk compared to iron therapies. The use of oral and intravenous iron is an effective, tolerated therapy modality for IDA which can be overlooked based on the degree of anemia. <b>Study Design and Methods:</b> Plan, Do, Study, Act methodology was used for this single institution quality improvement initiative. The objective was to decrease the percentage of PRBC transfusions in all admitted IDA patients from a baseline of 72% to a target of 50% by December 2022 and to sustain for 12 months. Interventions consisted of multidisciplinary, evidence-based didactic education sessions and development of a single institution clinical practice guideline for the treatment of IDA. <b>Results:</b> In the pre-education/baseline group, 72% (<i>n</i> = 57/79) of patients received PRBC transfusion for the treatment of IDA, compared to the posteducation/intervention group where 38% (<i>n</i> = 29/76) of patients received PRBC transfusion for the treatment of IDA (<i>p</i> value < 0.0001). In the pre-education/baseline group, 19% (<i>n</i> = 11/57) of patients received PRBC transfusions not indicated based on the developed CPG, compared to 6.9% (<i>n</i> = 2/18) in the posteducation/intervention group (<i>p</i> value = 0.20). <b>Discussion:</b> This work demonstrates how multidisciplinary, education- and evidence-based interventions lead to clinically and statistically significant reductions in PRBC transfusion for admitted patients with IDA.</p>","PeriodicalId":7325,"journal":{"name":"Advances in Hematology","volume":"2025 ","pages":"5513287"},"PeriodicalIF":0.0,"publicationDate":"2025-06-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12187434/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144482873","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 : 2025-05-19eCollection 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":"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}