Journal of Blood Medicine最新文献

{"title":"A Delphi Consensus by Hematologists on the Burden of Treatment on Patients with Severe Hemophilia in Mexico.","authors":"Jaime García Chávez, Berenice Sánchez Jara, María Teresa García Lee, Aída Mashenka Moreno González, Cecilia Rodríguez Castillejos, Laura Villarreal Martinez, Karla Maldonado Silva, Atenas Villela Peña","doi":"10.2147/JBM.S591589","DOIUrl":"10.2147/JBM.S591589","url":null,"abstract":"<p><strong>Purpose: </strong>Current treatment of hemophilia still represents an important burden for patients and caregivers. As a consequence, adherence can be poor, thus reducing efficiency of treatment. Then, a modified Delphi method was used to assess the burden of hemophilia treatment in Mexico.</p><p><strong>Methods: </strong>In the first round, the experts answered online a questionnaire with 32 statements structured in eight sections: overall efficacy of recombinant coagulation factors, delivery of recombinant factors, adherence to treatment with recombinant factors, emotional burden, physical load, interference with daily life, impact of adherence on efficacy, and satisfaction with treatment. Each statement was qualified from \"Totally disagree\" to \"Totally agree\". In the second round, those statements with less than 70% agreement between experts were discussed.</p><p><strong>Results: </strong>Consensus was reached in more than half of the questions. There was a consensus on the limited efficacy of current treatments, the complexity of preparation and administration of the recombinant coagulation factors, and the negative effect of the emotional burden, among other statements. Answers to the section related to the impact of adherence on efficacy showed the concern of experts about this issue.</p><p><strong>Conclusion: </strong>The burden of prophylactic hemophilia treatment on patients and caregivers in Mexico is substantial. Its negative effects on adherence and efficacy of treatment require evaluation of new approaches to hemophilia treatment.</p>","PeriodicalId":15166,"journal":{"name":"Journal of Blood Medicine","volume":"17 ","pages":"591589"},"PeriodicalIF":2.1,"publicationDate":"2026-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13291657/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148329751","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":"A Clinical Investigation of Hypoxic Red Blood Cell Administration in Patients with Transfusion-Dependent Hematological Malignancies and Burns.","authors":"Håkon Reikvam, Turid Helen Felli Lunde, Einar Klæboe Kristoffersen, Laurel Omert, Jill Bagdasarian, Stian K Almeland","doi":"10.2147/JBM.S573232","DOIUrl":"10.2147/JBM.S573232","url":null,"abstract":"<p><strong>Purpose: </strong>Red blood cell (RBC) transfusion is frequently given for patients with chronic anemias or acute bleeding after injury or surgery. A CE mark-certified and Food and Drug Administration authorized storage system (Hemanext ONE^®; Hemanext Inc. Lexington, MA, United States) has been developed to process and store leukocytes-reduced, O₂/CO₂ reduced RBCs under hypoxic conditions to improve RBC quality for transfusion. This prospective, open-label, single-center pilot safety study investigated the safety of hypoxic RBCs in 2 patient groups: hematological malignancies and acute burns.</p><p><strong>Patients and methods: </strong>The primary outcome was the number of patients who experienced an adverse event (AE) within 24 hours of transfusion and overall up to 7 days (±1 day) post-transfusion. Secondary outcomes included changes in hemoglobin (Hb) levels post-transfusion.</p><p><strong>Results: </strong>Twenty patients (10 with hematological malignancies, 10 with surgical bleeding during burn excision) aged 27-96 years were included (17 males; 8 with hematological malignancies, 9 with burns and 3 females; 2 with hematological malignancies, 1 with burns). All patients in the hematological malignancies group received 2 units of hypoxic RBCs. Patients in the burn group received a mean ±standard deviation (SD) of 1.8 ±0.4 units of hypoxic RBCs. No AEs related to hypoxic RBC transfusion were reported. The mean ±SD changes in Hb from baseline to 15-60 minutes post-transfusion were 1.2 ±0.5 g/dL (hematological malignancies group) and 0.3 ±1.0 g/dL (burn group).</p><p><strong>Conclusion: </strong>This cohort analysis showed that transfusion with hypoxic RBCs processed with the Hemanext ONE storage system was safe and well tolerated in both cohorts.</p>","PeriodicalId":15166,"journal":{"name":"Journal of Blood Medicine","volume":"17 ","pages":"573232"},"PeriodicalIF":2.1,"publicationDate":"2026-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13271058/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148271681","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":"ATG Plus Post-Transplantation Cyclophosphamide Improves EBV-DNA Clearance and GVHD in Patients of Adult Chronic Active Epstein-Barr Virus Disease Undergoing Allogeneic HSCT.","authors":"Min Liu, Qiuxia Yu, Yang Cao, Yaxian Ma, Zetong Hong, Qing Yin, Yan Feng, Tongjuan Li, Yicheng Zhang, Miao Zheng","doi":"10.2147/JBM.S593133","DOIUrl":"10.2147/JBM.S593133","url":null,"abstract":"<p><strong>Background: </strong>Chronic active Epstein-Barr virus disease (CAEBVD) faces a challenging prognosis due to its inflammatory and tumorigenic nature and lack of standard treatment. The outcomes of adults are more disheartening. We aim to elucidate the efficacy of allogeneic hematopoietic stem cell transplantation (allo-HSCT) in adult CAEBVD, while clarifying whether the combination of antithymocyte globulin (ATG) and post-transplant cyclophosphamide (PTCy) can improve survival and reduce transplant-related complications.</p><p><strong>Methods: </strong>We conducted a retrospective study analyzing adult CAEBVD patients who underwent allo-HSCT sequentially in the Department of Hematology at Tongji Hospital, Huazhong University of Science and Technology, from June 2015 to January 2022.</p><p><strong>Results: </strong>Of 20 patients included. The median age at diagnosis was 30.5 years, and the median follow-up time post-transplantation was 26.9 months. The 5-year overall survival (OS), progression-free survival (PFS), GVHD-free and relapse-free survival (GRFS), and non-relapse mortality (NRM) rates were 54.5%, 39.4%, 44.4%, and 37.4%, respectively. These data were similar in ATG alone (50.0%, 33.3%, 33.3%, and 40.0%) and ATG+PTCy (56.3%, 41.7%, 49.0%, and 37.5%). On multivariable Cox regression, pre-transplant hemophagocytic syndrome (25.0% vs. 75.0%, <i>P</i>=0.029) and higher EBV copies (25% vs. 61.9%, <i>P</i>=0.048) reduced the OS. On multivariable logistic regression, ATG+PTCy showed a lower incidence of grade II-IV acute GVHD (14.3% vs. 66.7%, <i>P</i>=0.037) and chronic GVHD (14.3% vs. 66.7%, <i>P</i>=0.037). Additionally, faster EBV clearance (P=0.002) and lower EBV reactivation (<i>P</i>=0.018) were observed in ATG+PTCy.</p><p><strong>Conclusion: </strong>Allo-HSCT can facilitate long-term survival in approximately half of adult CAEBVD. ATG+PTCy regimen did not improve survival but demonstrated advantages over ATG alone in reducing GVHD, eliminating EBV, and decreasing EBV reactivation.</p>","PeriodicalId":15166,"journal":{"name":"Journal of Blood Medicine","volume":"17 ","pages":"593133"},"PeriodicalIF":2.1,"publicationDate":"2026-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13242839/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148205069","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":"Care Patterns, Outcomes, and Costs in Peripheral T-Cell Lymphoma Patients with First-Line Treatment in Routine Clinical Practice in Europe.","authors":"Fjoralba Kristo, Sneha S Kelkar, Rutika Raina, Jordana K Schmier, Mehul R Dalal, Ajibade O Ashaye","doi":"10.2147/JBM.S553046","DOIUrl":"10.2147/JBM.S553046","url":null,"abstract":"<p><strong>Purpose: </strong>Limited real-world data exist on care patterns in peripheral T-cell lymphoma (PTCL) patients. We report care patterns and outcomes among PTCL patients in France, the United Kingdom (UK), Germany, and Italy in the first-line setting.</p><p><strong>Patients and methods: </strong>A retrospective chart review was undertaken for adults with newly diagnosed PTCL. Eligible patients received ≥1 line of therapy (LOT) and had clinical data for ≥1 year after diagnosis or until death. Outcomes included treatments, best response at the end of 1LOT, progression-free survival (PFS) and overall survival (OS).</p><p><strong>Results: </strong>Data from 195 patients (France=53; UK=56; Germany=56; Italy=30) were collected. Median age at diagnosis was 62.0 years, with 73.3% at stage III/IV. The most common histological subtype was PTCL-not otherwise specified (36.9%). For 1LOT, 49.7% and 36.4% of patients received CHOP and CHOEP regimens, respectively. A complete response was reported in 59.7% of patients. Median PFS from 1LOT initiation was 49.4 months; median OS was 77.1 months. First-line treatment costs were highest in the UK. The cost drivers were adjunctive therapy in France and Germany; stem cell transplant and hospitalization in the UK and Italy, respectively.</p><p><strong>Conclusion: </strong>This analysis improves the understanding of treatment outcomes and costs in PTCL patients in Europe.</p>","PeriodicalId":15166,"journal":{"name":"Journal of Blood Medicine","volume":"17 ","pages":"553046"},"PeriodicalIF":2.1,"publicationDate":"2026-05-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13228867/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148156601","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":"Malignancy-Associated Hemophagocytic Lymphohistiocytosis: An Experience of 15 Years in Polish Pediatric Hematology Centers.","authors":"Magdalena Wolowiec, Katarzyna Drabko, Lucyna Maciejka-Kemblowska, Ninela Irga-Jaworska, Katarzyna Smalisz, Szymon Skoczen, Renata Tomaszewska, Tomasz Szczepanski, Katarzyna Babol-Pokora, Wojciech Mlynarski, Katarzyna Popko, Urszula Demkow, Konrad Furmanczyk, Iwona Malinowska","doi":"10.2147/JBM.S571561","DOIUrl":"10.2147/JBM.S571561","url":null,"abstract":"<p><strong>Background: </strong>M-HLH is a type of HLH that most often occurs in children in the course of hematological malignancies. The diagnosis is based on meeting the criteria of the HLH-2004 Protocol. The disease has a rapid course, and the lack of proper diagnosis and treatment threatens the patient's life.</p><p><strong>Methods: </strong>The paper presents the thorough clinical characteristics, course of treatment, and treatment results in 23 patients treated in pediatric hemato-oncology centers in Poland and reported to Polish HLH registry. HLH diagnostic criteria, CNS involvement, coexisting viral infections, genetic test results, treatment modalities, therapeutic responses, relapse incidence, and final outcomes were analyzed. Clinical and laboratory factors were subjected to statistical analysis. Overall survival was estimated using the Kaplan-Meier method for the entire cohort and separately for subgroups defined by type of malignancy and treatment strategy. Separate univariable Cox proportional hazards models were used to explore the association between selected clinical factors and survival.</p><p><strong>Results: </strong>The median age at diagnosis was 11.7 years, in 8 patients HLH symptoms preceded the cancer diagnosis. Acute leukemia was the factor causing M-HLH in 12 patients, in the remaining patients it was lymphoma. The treatment included tumor-directed therapy with or without HLH-directed therapy. The probability of survival in the study group was 0.539 (95% CI 0.364-0.798). Remission of HLH was statistically significant (p-value = 0.0007) and reduced the risk of death by 90% (HR=0.10).</p><p><strong>Conclusion: </strong>Quick and precise diagnosis and implementation of appropriate treatment bringing the patient into HLH remission is extremely important for survival.</p>","PeriodicalId":15166,"journal":{"name":"Journal of Blood Medicine","volume":"17 ","pages":"571561"},"PeriodicalIF":2.1,"publicationDate":"2026-05-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13222552/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148143291","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":"Hairy Cell Leukemia: Clinical Characteristics and Outcomes from a Single Center in the Middle East and North Africa.","authors":"Shehab Mohamed, Mohamad Wajeh Dulli, Abdulrahman Al-Mashdali, Rola Ghasoub, Mohammed Abdulgayoom, Honar Cherif, Susanna Akiki, Anil Elahie, Deena Mudawi, Hesham Elsabah, Mohamed Yassin, Noor Salah Moustafa, Leen Haj Saleh, Priyadarshini Asmita Vatsayana, Samah Kohla, Firyal Ibrahim, Dina Soliman","doi":"10.2147/JBM.S595093","DOIUrl":"10.2147/JBM.S595093","url":null,"abstract":"<p><strong>Purpose: </strong>Hairy Cell Leukemia (HCL) is a rare, chronic B-cell lymphoproliferative neoplasm characterized by the accumulation of abnormal lymphocytes in the bone marrow and spleen. Although outcomes are generally favorable with current therapies, data from diverse geographic and ethnic populations remain limited. This study highlights the disease clinicopathologic characteristics, treatment, and outcome within one center in the Middle East.</p><p><strong>Methods: </strong>A retrospective analysis was conducted on patients diagnosed with HCL at a tertiary care center, meeting the 5th edition WHO and ICC 2022 diagnostic criteria. Clinical, laboratory, and pathological data were extracted from institutional records.</p><p><strong>Results: </strong>Twenty-two patients were identified. The cohort represented diverse nationalities across Asia, Africa, Europe, and North America. Most cases were detected incidentally, while others presented with constitutional symptoms, abdominal pain, or febrile neutropenia. All but one case were classic HCL. Most expressed Pan B-cell markers, though a few showed aberrant loss of CD19, PAX-5, or CD103. The BRAF V600E mutation was detected in 82% of the tested cases. Treatment regimens included Cladribine alone or with Rituximab, yielding high response rates and minimal toxicity. Five-year overall survival was 100%.</p><p><strong>Conclusion: </strong>In this multiethnic Middle Eastern and North African cohort, HCL predominantly affected middle-aged men and was often incidentally detected. Cladribine-based therapy achieved durable remissions with excellent survival, highlighting consistent efficacy across diverse populations.</p>","PeriodicalId":15166,"journal":{"name":"Journal of Blood Medicine","volume":"17 ","pages":"595093"},"PeriodicalIF":2.1,"publicationDate":"2026-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13200198/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148030700","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":"Catheter-Related Superficial Venous Thrombosis of the Upper Extremity: A Narrative Review of Current Evidence, Diagnostic Pathways, and Management Strategies.","authors":"Carmine Siniscalchi, Manuela Basaglia, Tiziana Meschi, Nicoletta Cerundolo, Pierpaolo Di Micco","doi":"10.2147/JBM.S601961","DOIUrl":"10.2147/JBM.S601961","url":null,"abstract":"<p><p>Catheter-related superficial venous thrombosis (SVT) of the upper extremity is a frequent complication of peripheral venous cannulation and longer peripheral devices such as midline catheters and peripherally inserted central catheters (PICCs). Although historically regarded as a benign and self-limited condition, upper-extremity SVT is increasingly recognized as a clinically meaningful entity within a continuum that includes chemical or mechanical phlebitis, extensive superficial thrombophlebitis, catheter-related deep vein thrombosis (UEDVT), septic thrombophlebitis, and rarely pulmonary embolism. Contemporary practice remains heterogeneous, largely because high-quality evidence supporting specific antithrombotic strategies in catheter-provoked upper-extremity SVT is limited. Duplex ultrasonography is central to modern management, as it confirms the diagnosis, quantifies thrombus extension, defines proximity to the deep venous system, and rules out concomitant UEDVT. Available data suggest that many patients with limited cannula-provoked SVT can be managed conservatively after catheter removal and symptomatic therapy, whereas patients with active cancer, extensive thrombosis, progression, or proximity to the axillary subclavian system may warrant anticoagulation on an individualized basis. Septic thrombophlebitis requires urgent catheter removal, blood cultures, targeted intravenous antibiotics, and multidisciplinary management. Prevention through vascular access stewardship, optimal device selection, and attention to catheter-to-vein ratio is likely the most impactful approach to reduce the burden of disease.</p>","PeriodicalId":15166,"journal":{"name":"Journal of Blood Medicine","volume":"17 ","pages":"601961"},"PeriodicalIF":2.1,"publicationDate":"2026-05-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13179760/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147963924","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":"Teclistamab-Induced Localized Pleural Cavity Cytokine Release Syndrome in a Multiple Myeloma Patient Managed with Intrapleural Dexamethasone Administration.","authors":"Qian Luo, Xuzhao Zhang, Xi Qiu, Huawei Jiang, Yang Xu","doi":"10.2147/JBM.S588402","DOIUrl":"10.2147/JBM.S588402","url":null,"abstract":"<p><p>Teclistamab, a B-cell maturation antigen (BCMA) × CD3 bispecific T-cell engager, has shown significant efficacy in patients with refractory multiple myeloma (MM). However, the clinical characteristics and optimal management strategies for localized cytokine release syndrome (CRS) remain poorly defined compared with systemic CRS. We report the case of a 57-year-old woman with refractory MM who developed both systemic and localized CRS following teclistamab therapy. She initially presented with grade 3 systemic CRS during step-up dosing phase, which partially resolved with intravenous methylprednisolone. However, interleukin-6 remained elevated in pleural effusions but not in serum, indicating localized pleural cavity CRS. Management with pleural fluid drainage combined with intrapleural dexamethasone successfully controlled the localized CRS. At two months, the patient achieved a partial response and continued teclistamab therapy. To our knowledge, this is among the first reported cases of teclistamab-associated localized pleural cavity CRS effectively managed with intrapleural dexamethasone.</p>","PeriodicalId":15166,"journal":{"name":"Journal of Blood Medicine","volume":"17 ","pages":"588402"},"PeriodicalIF":2.1,"publicationDate":"2026-04-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13116515/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147771915","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":"Characterization of Inherited Bleeding Disorders in Egyptian Children in a Tertiary Care Center: A 10 Years Experience.","authors":"Mona El-Ghamrawy, Marwa Abdelhady, Salma Mohamed Fathy Zahran, Mohamed Salah Eldin Mohamed Abdel Kader","doi":"10.2147/JBM.S604537","DOIUrl":"10.2147/JBM.S604537","url":null,"abstract":"<p><strong>Background: </strong>Inherited bleeding disorders (IBDs) require accurate diagnosis and long-term management. This study characterized the clinical and hematologic profile of Egyptian children with IBDs managed at Cairo University Children's Hospital and Misr University for Science and Technology.</p><p><strong>Methods: </strong>This retrospective longitudinal observational study included 200 pediatric patients with inherited coagulation or platelet disorders followed between 2015 and 2025. Data collected included demographics, family history, bleeding manifestations, complications, treatment exposure, functional scores, imaging findings, and confirmatory laboratory investigations.</p><p><strong>Results: </strong>Hemophilia A (HA) and von Willebrand disease (vWD) were the most common disorders, accounting for 32.0% and 28.5% of cases, respectively. Among rare inherited coagulation defects, fibrinogen disorders and factor VII deficiency were the most frequent. HA showed the highest hospitalization rate, annual bleeding rate, and ISTH bleeding score, while joint disease was most prominent in hemophilia. Intracranial hemorrhage occurred most often in factor VII deficiency. In HA, the Functional Independence Score of Hemophilia (FISH) was the best discriminator of chronic hemophilic arthropathy (AUC 0.715; cutoff ≤ 26), followed by annual bleeding rate (AUC 0.695; cutoff > 6/year). Persistent high-titer inhibitors developed in 17.2% of HA patients. Most vWD cases were type 1 (75.4%), and Glanzmann thrombasthenia was the most common inherited platelet disorder.</p><p><strong>Conclusion: </strong>Egyptian children with IBDs show heterogeneous clinical presentations and outcomes. Severe phenotypes, particularly HA and type 3 vWD, were associated with earlier bleeding onset, greater morbidity, and the need for individualized management.</p>","PeriodicalId":15166,"journal":{"name":"Journal of Blood Medicine","volume":"17 ","pages":"604537"},"PeriodicalIF":2.1,"publicationDate":"2026-04-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13110028/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147771830","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":"From Splenectomy to Partial Splenic Embolization, Which is Better for Hereditary Spherocytosis?","authors":"Mengyuan Si, Shanghong Yang, Zijian Chen, Aixia Dou","doi":"10.2147/JBM.S595335","DOIUrl":"10.2147/JBM.S595335","url":null,"abstract":"<p><p>Hereditary Spherocytosis (HS), with characteristic red blood cells (RBCs), is a group of genetic hemolytic disorders that occur in an autosomal-dominant manner. It is clinically characterized by anemia, splenomegaly, jaundice, and mutations in key genes. Splenectomy is the standard treatment for HS. Alternatively, partial splenic embolization (PSE) balances efficacy and safety, making it a promising solution. To determine how to choose surgery and ensure correct management, we reviewed the indications, techniques, efficacies, complications, and progress of alternative solutions to reach a comprehensive conclusion. Core clinical dimensions between Splenectomy and PSE in the treatment of HS, including surgical indications, operative techniques, hemoglobin improvement, infection risk, immune function preservation, long-term complications and recurrence rate. PSE is better than Splenectomy. Furthermore, forefront-targeted drugs, precise embolization ranges, and multidisciplinary team (MDT) discussions were reviewed. Collaboration requires multiple departments and family members. Through this narrative review, we hope to provide practical advice for long-term follow-up and emergency management.</p>","PeriodicalId":15166,"journal":{"name":"Journal of Blood Medicine","volume":"17 ","pages":"595335"},"PeriodicalIF":2.1,"publicationDate":"2026-04-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13105199/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147771897","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}