Hematology Reports最新文献

{"title":"The Real-World Outcomes of Relapsed/Refractory Multiple Myeloma Treated with Elotuzumab, Pomalidomide, and Dexamethasone.","authors":"Hitomi Nakayama, Yoshinobu Aisa, Chisako Ito, Aki Sakurai, Tomonori Nakazato","doi":"10.3390/hematolrep16040058","DOIUrl":"https://doi.org/10.3390/hematolrep16040058","url":null,"abstract":"<p><p><b>Introduction</b>: A combination of elotuzumab, pomalidomide, and dexamethasone (EPd) was approved for the treatment of relapsed/refractory multiple myeloma (RRMM) following the ELOQUENT-3 phase II clinical trial. However, the clinical experience with this therapy is still limited. In this retrospective study, we analyzed the efficacy and safety of EPd in a real-world cohort of RRMM patients. <b>Patients and Methods</b>: The medical records of 22 patients who received EPd for RRMM at Yokohama Municipal Citizen's Hospital (Japan) between January 2020 and July 2021 were reviewed. <b>Results</b>: The median age of our cohort was 73.5 years. The overall response rate was 55%. With a median follow-up of 20.2 months, the median progression-free survival (PFS) was 9.1 months (95% confidence interval [CI], 2.5-23.0 months). The median PFS was shorter in patients with a poor performance status (PS) than in those with favorable PS (2.5 vs. 10.8 months; <i>p</i> < 0.01). Patients with prior daratumumab had significantly shorter PFS than those without prior daratumumab (2.1 vs. 23.0 months; <i>p</i> < 0.01). Additionally, patients with prior pomalidomide had significantly shorter PFS (1.7 vs. 10.3 months; <i>p</i> < 0.01). In the multivariate analysis, poor PS (hazard ratio [HR] = 4.1, 95% CI: 1.1-15.6; <i>p</i> = 0.04) and prior exposure to daratumumab (HR = 3.8, 95% CI: 1.1-13.8; <i>p</i> = 0.04) remained significantly associated with shorter PFS. <b>Conclusions</b>: The results of our study suggest that EPd is an active and well-tolerated regimen in RRMM, even in real-world patients. Furthermore, EPd may be useful, especially in daratumumab-naïve patients.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"16 4","pages":"593-602"},"PeriodicalIF":1.1,"publicationDate":"2024-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11503276/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142499162","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":"Two Cases of Immune Thrombocytopenia (ITP) Related to Viral Vector Vaccination ChAdOx1-S (AstraZeneca) and a Good Response after Thrombopoietin Receptor Agonist (TPO-RA) Therapy.","authors":"Konstantina Salveridou, Theodoros Tzamalis, Maika Klaiber-Hakimi, Sabine Haase, Stefanie Gröpper, Aristoteles Giagounidis","doi":"10.3390/hematolrep16040057","DOIUrl":"https://doi.org/10.3390/hematolrep16040057","url":null,"abstract":"<p><strong>Background: </strong>In 2019, a new coronavirus disease emerged in Wuhan, China, known as SARS-CoV-2, severe acute respiratory syndrome coronavirus 2, and caused an ongoing pandemic. Symptomatology of the syndrome is variable, with complications extending to hematopoiesis and hemostasis. Approximately a year after onset of the virus, four vaccination formulas became available to the public, based on a viral vector or mRNA technology. These vaccine formulas have been hampered with hematological complications, like vaccine-induced immune thrombotic thrombocytopenia (VITT) and vaccine-related ITP (immune thrombocytopenic purpura). ITP is a disease with autoimmune pathogenesis characterized by antibody production against platelets and an increased hemorrhagic risk. A decent number of cases have been referred to as possible adverse effects of COVID-19 vaccinations.</p><p><strong>Case presentation: </strong>in this case report, we present two cases of newly diagnosed ITP after vaccination with ChAdOx1-S (AstraZeneca), with a good response to treatment with thrombopoietin-receptor agonists (TPO-RAs).</p><p><strong>Discussion: </strong>we observed an absence of response after corticosteroids and IVIG therapy and a positive therapeutic outcome on TPO-RA.</p><p><strong>Conclusions: </strong>in the ongoing pandemic, there is an urgent need to create therapeutic guidelines for vaccination-related clinical entities and to clarify indications for the vaccination of patients with pre-existing hematological diseases.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"16 4","pages":"585-592"},"PeriodicalIF":1.1,"publicationDate":"2024-09-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11503447/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142499164","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":"Complete Remission with Inotuzumab Ozogamicin as Fourth-Line Salvage Therapy in a Child with Relapsed/Refractory Acute Lymphoblastic Leukemia.","authors":"Athanasios Tragiannidis, Vassiliki Antari, Eleni Tsotridou, Theodoros Sidiropoulos, Aikaterini Kaisari, Maria Palabougiouki, Timoleon-Achilleas Vyzantiadis, Emmanuel Hatzipantelis, Assimina Galli-Tsinopoulou, Evgenios Goussetis","doi":"10.3390/hematolrep16040056","DOIUrl":"https://doi.org/10.3390/hematolrep16040056","url":null,"abstract":"<p><p><b>Background:</b> Despite the progress achieved regarding survival rates in childhood acute lymphoblastic leukemia (ALL), relapsed or refractory disease still poses a therapeutic challenge. Inotuzumab ozogamicin is a CD22-directed monoclonal antibody conjugated to calicheamicin, which has been approved by the Food and Drug Administration for adults and pediatric patients 1 year and older with relapsed or refractory CD22-positive B-cell precursor acute lymphoblastic leukemia. <b>Case presentation:</b> Herein, we present the case of a 23-month-old girl with high-risk B-ALL who experienced very early isolated medullary relapse; following the failure of conventional chemotherapy according to the ALL-IC REL 2016 protocol, she went on to receive the bispecific T-cell engager (BiTE) blinatumomab and subsequently, due to refractory disease, the combination of fludarabine, cytarabine, and the proteasome inhibitor bortezomib without achieving remission. Given the high CD22 expression by the lymphoblasts, off-label use of inotuzumab ozogamicin (InO) was chosen and administrated in a 28-day cycle as a salvage treatment. The minimal residual disease (MRD) was 0.08% on day 28, and InO was continued, thus achieving MRD negativity; the patient successfully underwent an allogeneic stem cell transplantation from a matched family donor. <b>Conclusions:</b> Our case highlights the efficacy and safety of InO as a salvage treatment in the setting of relapsed B-ALL refractory not only to conventional chemotherapy but also to novel treatments, such as blinatumomab and bortezomib.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"16 4","pages":"579-584"},"PeriodicalIF":1.1,"publicationDate":"2024-09-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11503335/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142499156","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":"Padua Prediction Score and Hospital-Acquired Proximal and Isolated Distal Deep Vein Thrombosis in Symptomatic Patients.","authors":"Michelangelo Sartori, Miriam Fiocca, Mario Soldati, Laura Borgese, Elisabetta Favaretto, Benilde Cosmi","doi":"10.3390/hematolrep16040055","DOIUrl":"https://doi.org/10.3390/hematolrep16040055","url":null,"abstract":"<p><strong>Background: </strong>Hospital-acquired deep vein thrombosis (DVT) is an important cause of morbidity and mortality.</p><p><strong>Objectives: </strong>The purpose of this study was to evaluate the prevalence of proximal lower limb DVT and isolated distal DVT (IDDVT) and their relationship to the Padua Prediction Score (PPS) in acutely ill, hospitalized patients.</p><p><strong>Methods: </strong>In a single-center cross-sectional study, all inpatients from medical departments with suspected lower-extremity DVT were evaluated with whole-leg ultrasonography during 183 days from 2016 to 2017.</p><p><strong>Results: </strong>Among the 505 inpatients (age 78.0 ± 13.3, females 59.2%) from medical departments, 204 (40.2%) had PPS ≥ 4, but only 54.4% of them underwent pharmacological thrombo-prophylaxis. Whole-leg ultrasonography detected 47 proximal DVTs (9.3%) and 65 IDDVTs (12.8%). Proximal DVT prevalence was higher in patients with high PPS vs. those with low PPS (12.7% vs. 7.0% <i>p</i> = 0.029, respectively), whereas IDDVT prevalence was similar in patients with high and low PPS (14.7% vs. 11.6% <i>p</i> = 0.311, respectively). The area under the receiver operating curve (AUC) for the PPS was 0.62 ± 0.03 for all DVTs, 0.64 ± 0.04 for proximal DVTs, and 0.58 ± 0.04 for IDDVTs.</p><p><strong>Conclusions: </strong>In hospitalized patients, IDDVT had similar prevalence regardless of PPS risk stratification. Adherence to thrombo-prophylaxis in patients was still far from optimal.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"16 4","pages":"568-578"},"PeriodicalIF":1.1,"publicationDate":"2024-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11503401/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142499160","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":"How We Treat Hemolytic Anemia Due to Pyruvate Kinase Deficiency.","authors":"Sara Tama-Shekan, Valeria Moreno, Ludovic Saba, Chakra P Chaulagain","doi":"10.3390/hematolrep16030054","DOIUrl":"10.3390/hematolrep16030054","url":null,"abstract":"<p><strong>Background: </strong>Pyruvate kinase (PK) deficiency is an inherited red blood cell (RBC) enzyme disorder that results in non-immune chronic hemolytic anemia. Characteristic symptoms of PK deficiency include anemia, fatigue, splenomegaly, jaundice, gallstones, thrombosis, and transfusional iron overload. Previously, treatments aimed at symptomatic management with RBC transfusions, phototherapy, folic acid supplementation, splenectomy, and iron chelation therapy when iron overload was documented. Mitapivat, a recently approved medication for treatment of PK-deficiency hemolytic anemia, is an oral allosteric activator of wild-type and mutant RBC PK enzymes. In this paper, we describe three cases of PK-deficiency anemia treated with mitapivat and describe modern management of this rare hemolytic disorder.</p><p><strong>Methods: </strong>A retrospective healthcare database analysis was conducted to extract relevant information. Both quantitative and qualitative methods were integrated to provide a more comprehensive understanding of the cases.</p><p><strong>Results: </strong>Two patients responded well to treatment with mitapivat, noted by an increase in hemoglobin levels, improvements in hemolytic markers, less frequent or no RBC transfusion requirements, and improvements in fatigue. One patient carrying two non-missense mutations of the <i>PKLR</i> gene did not respond to treatment with mitapivat. As variations in patient-specific factors (including genotype) can lead to different clinical manifestations and responses to treatment, we recommend considering both the phenotype (clinical symptoms and signs) and the genotype of the <i>PKLR</i> gene when making therapeutic decisions about starting a patient on mitapivat.</p><p><strong>Conclusions: </strong>While mitapivat addresses the previously unmet needs of most patients with PK deficiency as the first and only disease-modifying medication to receive approval for this condition, not all patients with PK deficiency are amenable to treatment with mitapivat.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"16 3","pages":"559-567"},"PeriodicalIF":1.1,"publicationDate":"2024-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11417781/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142284430","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":"Hyperbaric Oxygen Therapy during Pregnancy for Critical Anemia Secondary to Sickle Cell Disease with Post-Transfusion Hyperhemolysis: A Case Report.","authors":"Shawn Khan, Connor T A Brenna, Jacob Pendergrast, A Kinga Malinowski, Marcus Salvatori, Rita Katznelson, Jordan Tarshis","doi":"10.3390/hematolrep16030053","DOIUrl":"10.3390/hematolrep16030053","url":null,"abstract":"<p><p><b>Background:</b> Sickle cell disease is the most common human monogenetic disease, and its risks are amplified during pregnancy. <b>Methods</b>: This report describes a 35-year-old woman with HgbSS sickle cell disease who developed hyperhemolysis syndrome after undergoing an exchange transfusion during pregnancy. <b>Results:</b> In addition to conventional medical treatment, the patient received prepartum hyperbaric oxygen therapy (HBOT), totaling 17 treatments for the indication of severe anemia. She experienced significant clinical improvement while undergoing HBOT and ultimately delivered a healthy preterm infant by cesarean section. <b>Conclusions:</b> The risks, benefits, and challenges of using HBOT in this unique context are discussed.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"16 3","pages":"552-558"},"PeriodicalIF":1.1,"publicationDate":"2024-08-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11417712/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142284431","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":"IgG-k/IgG-λ Para-Osseous Plasmacytoma Relapsed as Soft-Tissue Plasmacytoma with IgA-k Immunophenotype: A Case Report and Review of the Literature on Related Biochemical Aspects.","authors":"Manlio Fazio, Chiara Maria Catena Sorbello, Vittorio Del Fabro, Alessandra Romano, Maria Teresa Cannizzaro, Nunziatina Laura Parrinello, Benedetta Esposito, Sara Frazzetto, Federica Elia, Francesco Di Raimondo, Concetta Conticello","doi":"10.3390/hematolrep16030052","DOIUrl":"10.3390/hematolrep16030052","url":null,"abstract":"<p><p>Neoplastic plasma cells (PCs) proliferation at anatomic sites dislocated from the bone marrow (BM) or their contiguous growth from osseous lesions that disrupt the cortical bone is termed extramedullary multiple myeloma (EMD). EMD still remains challenging from a therapeutic and biological perspective. Pathogenesis has not been completely clarified, and it is generally associated with high-risk cytogenetics (HRCAs). In order to emphasize the clinical and biochemical complexity of this disease, we have decided to describe the case of a patient affected by relapsed-refractory (RR) EMD, which presented as para-osseous plasmacytoma with a bi-phenotypical immunoglobulin (Ig) component and lately relapsed as soft-tissue plasmacytoma with a total immunophenotype switch. We have also hypothesized a correlation between Ig patterns and prognosis and suggested the possible inclusion of these biochemical features in the general risk assessment.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"16 3","pages":"541-551"},"PeriodicalIF":1.1,"publicationDate":"2024-08-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11417817/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142284441","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":"First-Line Combination with Proteasome Inhibitor-Based Treatment and Zoledronic Acid Is Effective in Reducing Later Fractures in Multiple Myeloma Irrespective of Multiple Myeloma Bone Disease at Diagnosis.","authors":"Veera Eskelinen, Elise Nivakoski, Kirsi Launonen, Anu Partanen, Sakari Kakko, Milla E L Kuusisto","doi":"10.3390/hematolrep16030051","DOIUrl":"10.3390/hematolrep16030051","url":null,"abstract":"<p><p>The present study provides real-world evidence on the treatment of multiple myeloma (MM) bone disease with various bisphosphonates combined for different myeloma-specific treatments as no validated data regarding the best combination treatment for bone disease associated with MM are available. We examined retrospectively 345 MM patients treated with autologous stem cell transplantation in Finland during 1996-2020. The median age of the patients was 60 years with a median follow-up time of 50 months (1-339). At diagnosis, 72.1% of the patients had myeloma-associated bone disease and 45.8% had fractures. Most patients (58.8%) received proteasome inhibitor (PI)-containing treatment at first line. MM bone disease was treated in 91.6% of the patients; 49.9% received zoledronic acid (ZA) and 29.9% pamidronate. Inferior overall survival was associated with MM bone disease at diagnosis (<i>p</i> = 0.005) or a fracture at diagnosis (<i>p</i> = 0.003). A later fracture was identified in 29% of the patients, and in those patients without MM bone disease at diagnosis later fractures were less common after ZA treatment (<i>p</i> = 0.049). PI-based treatment plus ZA (<i>p</i> = 0.019) seemed to be the best combination to prevent later fractures, even though the same patient subgroup was more likely to experience relapse (<i>p</i> = 0.018), and also when excluding patients with previous induction therapy without novel agents (<i>p</i> = 0.008). To conclude, this study suggests that the best therapy to prevent later fractures in MM might be PI-based treatment combined with ZA.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"16 3","pages":"529-540"},"PeriodicalIF":1.1,"publicationDate":"2024-08-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11348108/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142072603","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":"Lymphocytic Lymphoma Transforming into Hodgkin Lymphoma in Sub-Saharan Africa: Case Report and Literature Review.","authors":"Sokhna Aïssatou Touré, Dibor Niang, Serigne Mourtalla Gueye, Mohamed Keita, Alioune Badara Diallo, Elimane Seydi Bousso, Fatma Dieng, Blaise Felix Faye, Moussa Seck, Saliou Diop","doi":"10.3390/hematolrep16030050","DOIUrl":"10.3390/hematolrep16030050","url":null,"abstract":"<p><p>The Hodgkin variant Richter syndrome (HvRS) is an infrequent complication occurring in 1% of lymphocytic lymphoma/chronic lymphocytic leukemia patients. We report a case of HvRS diagnosed in Sub-Saharan Africa. A 63-year-old patient was consulted for the investigation of an abdominal mass that had been evolving for 5 years prior to admission. His history revealed night sweats, 13% weight loss in 3 months and persistent pruritis. Examination revealed bilateral cervical axillary and inguinal macroadenopathies, painless abdominal distension, pruritic lesions and WHO 2 PS. The blood count showed anemia at 9.5 g/dL. Histology revealed a lymphomatous proliferation of diffuse architecture, nodular in places, with Hodgkin and Sternberg cells associated with small lymphocytes, histiocytes and eosinophilic polymorphs. Immunohistochemistry showed CD20, PAX5, BCL2, CD5, CD23 and MYC positivity; Ki67 at 10% and cyclin D1, BCL6 and CD10 negativity; CD30 positivity on Hodgkin and Sternberg cells that remained CD20 negative; difficulty interpreting CD15; EBV positivity (EBERs); and CD3 and CD5 positivity on reactive T cells. CD138 and kappa and lambda light chains were non-contributory. The extension work-up classified the patient as Ann Arbor stage III B with a Hasenclever score of 3/7. This case illustrates the difficulties in diagnosing HvRS in our countries, where the number of haematopathologists is insufficient and the technical facilities are limited.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"16 3","pages":"523-528"},"PeriodicalIF":1.1,"publicationDate":"2024-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11348024/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142072605","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":"Investigation of Delayed Transfusion Reactions in Sickle Cell Disease Patients Polytransfused in the Brazilian Amazon.","authors":"Lorena Alves Santos, Anne Cristine Gomes de Almeida, Andrea Monteiro Tarragô, Nina Rosa Gonçalves da Silva, Juliana Nascimento Vitoriano da Silva, Mônica Moura de Souza, Monik Oney Oliveira Nascimento, Marcelo Reis do Nascimento, Ana Caroline Dos Santos Castro, Cinthia Xerez de Albuquerque, Evilázio Cunha Cardoso, José Pereira Moura Neto, Sérgio Roberto Lopes Albuquerque","doi":"10.3390/hematolrep16030049","DOIUrl":"10.3390/hematolrep16030049","url":null,"abstract":"<p><strong>Background: </strong>Sickle cell disease (SCD) affects approximately 100,000 people in the United States and millions worldwide, with the highest prevalence of 70% of SCD being found in individuals of African ethnicity. Delayed hemolytic, alloimmunization, and anamnestic transfusion reactions in multiple transfusion patients need to be investigated and managed to avoid a worsening of the patient's clinical status.</p><p><strong>Objective: </strong>This paper aims to investigate delayed transfusion reactions in SCD patients who were polytransfused in the Brazilian Amazon.</p><p><strong>Material and methods: </strong>The clinical and laboratory indicators of SCD patients with more than four transfusions were investigated. The patients were treated at the Fundação Hospitalar de Hematologia e Hemoterapia do Estado do Amazonas, Brazil.</p><p><strong>Results: </strong>A total of 44 polytransfused patients with SCD were followed. Regarding Rh phenotype, it was possible to observe a frequency of 26.6% (12) patients with the RZRZ (DCE/DCE) phenotype, in addition to 4.5% (two) patients with <i>RH</i> and <i>RHCE</i> variants. It was also possible to observe 20.5% (nine) patients with an alloimmunization reaction, who presented the following alloantibodies: anti-RhD, anti-E, anti-K, anti-Jk^b, anti-N, anti-S, and anti-Di^a, two of which are unidentified. Of these, four (44.4%) patients also presented autoantibodies, anti-e, and three unidentified antibodies, and four (44.4%) patients presented an anamnestic reaction, with anti-RhD, K, and Jkb antibodies. Of the 44 patients monitored, 54.4% (24) had clinical and laboratory indicators of a delayed hemolytic reaction.</p><p><strong>Conclusion: </strong>Delayed transfusion reactions, often neglected, occur frequently. Therefore, transfusions need to be monitored for at least 28 days, with medical investigation of clinical and laboratory indicators to make greater use of this therapeutic resource.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"16 3","pages":"512-522"},"PeriodicalIF":1.1,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11348377/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142072604","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}