Diffuse large B-cell lymphoma (DLBCL) is the most common subtype of non-Hodgkin lymphoma. Hematologic malignancies are known precipitants of disseminated intravascular coagulation (DIC), and the coexistence of these conditions is associated with poor outcomes; however, large-scale data specifically examining concurrent DLBCL and DIC are limited. We performed a retrospective cohort study using the National Inpatient Sample (NIS) to evaluate in-hospital mortality among adult hospitalizations with DIC and/or DLBCL identified using ICD-10 codes. Adults aged ≥ 18 years with a diagnosis of DLBCL and/or DIC were included. In-hospital mortality among patients with concurrent DLBCL and DIC was 54.1%, significantly higher than mortality observed in patients with either condition alone (p < 0.001). In multivariable analysis restricted to patients with both DLBCL and DIC, independent predictors of increased mortality included HIV/AIDS (aOR 4.266, 95% CI: 1.886-9.649; p < 0.001), septic shock (aOR 3.201, 95% CI: 2.305-4.443; p < 0.001), mechanical ventilation (aOR 2.948, 95% CI: 2.175-3.995; p < 0.001), vasopressor use (aOR 2.759, 95% CI: 1.789-4.255; p < 0.001), chronic kidney disease (aOR 2.523, 95% CI: 1.596-3.989; p < 0.001), acute kidney injury (aOR 1.884, 95% CI: 1.347-2.637; p < 0.001), Black race (aOR 2.221, 95% CI: 1.418-3.477; p < 0.001), and Asian or Pacific Islander race (aOR 2.383, 95% CI: 1.329-4.271; p = 0.004) compared to White patients. Bleeding events, thromboembolic complications, stem cell transplantation, and CAR-T therapy were not independently associated with mortality. These findings demonstrate that among patients with DLBCL, the development of DIC is associated with markedly elevated in-hospital mortality, driven predominantly by critical illness, organ dysfunction, and comorbidity burden rather than bleeding or thrombosis alone. Racial disparities in outcomes were independently observed after adjusting for illness severity and comorbidities, warranting further investigation. Early recognition of DIC in patients with DLBCL and aggressive management of systemic complications, particularly septic shock, respiratory failure, and renal dysfunction, may help mitigate disease severity and improve outcomes.
Introduction:Due to limited supportive care, low- and middle-income countries balance delivering intensive, curative chemotherapy with the risk of treatment-related toxicity. Acute lymphoblastic leukaemia (ALL) is the commonest childhood cancer, and induction is the highest risk period for morbidity and mortality. The study describes induction toxicities among children with ALL at Korle Bu Teaching Hospital, Ghana. Methods:This hospital-based cohort study involved children aged 1-17 years newly diagnosed with ALL from August 2021 to January 2023. Demographic, clinical and laboratory data were recorded. Descriptive statistics (median [interquartile range]) were used to summarise continuous variables. Frequency and percentages were used to describe categorical variables. Toxicities were graded using the Common Terminology Criteria for Adverse Events, Version 5.0. Results:Thirty-one children were included. Median age was 5 years (interquartile range [IQR]: 3-10 years). All 31 had at least one induction toxicity of any grade, and 29 (93.4%) had at least one severe (Grade ≥ 3). The most common toxicities were anaemia, thrombocytopenia, sepsis, febrile neutropenia, mucositis and hypertension; bleeding, acute pancreatitis, seizures and hepatotoxicity were less common. Induction mortality rate was 19.3%. There was no association found between age, sex, initial white cell count, treatment protocol and development of any severe toxicity. Conclusion:Severe induction toxicities were common in our cohort with unacceptably high mortality, underscoring the need to prioritise enhanced bedside clinical monitoring for hypertension, improved access to blood products and optimal infection prevention and control practices. Further research exploring the relationship between induction toxicities and patient outcomes is essential.
The COVID-19 pandemic posed significant challenges for patients with hematological malignancies, including multiple myeloma (MM), who experienced increased hospitalization and mortality rates due to frailty and immune suppression. This retrospective, multicenter study aimed to evaluate the impact of antiviral therapies and COVID-19 vaccination doses on clinical outcomes in 206 symptomatic MM patients undergoing active treatment across five Italian regions. The majority (90%) received at least one vaccine dose, and 61% were treated with antiviral agents (nirmatrelvir/ritonavir, molnupiravir, or remdesivir). Of the cohort, 90% were vaccinated, with 89% receiving ≥ 3 doses, and 61% received antiviral agents (nirmatrelvir/ritonavir, molnupiravir, or remdesivir). Patients vaccinated with ≥ 3 doses showed significantly reduced infection severity (p = 0.044), hospitalization rates (p = 0.008), and infection resolution time (p = 0.037). Unvaccinated patients without antiviral therapy had a significantly higher mortality rate (p = 0.004). Antiviral therapy, especially when combined with ≥ 3 vaccine doses, further shortened infection resolution time (p = 0.011) but did not significantly affect hospitalization rates or disease severity. This study highlights the critical role of ≥ 3 vaccine doses combined with antiviral therapy in improving COVID-19 outcomes in MM patients, with a reduced infection duration and lower progression to severe disease. Further investigation is required to optimize treatment strategies in this vulnerable population.
Introduction Sickle cell disease (SCD) is a genetic condition characterized by recurrent vaso‐occlusion and chronic hemolysis, which lead to long‐term irreversible complications, reducing patients’ quality of life and survival. Crizanlizumab is a humanized monoclonal antibody that acts in the pathogenesis of vaso‐occlusive crisis (VOCs) preventing their occurrence. The objective of this study is to describe the effectiveness and safety of crizanlizumab in Brazilian patients with SCD. We conducted a retrospective, multicenter study based on medical chart data. Materials and Methods The study period encompassed 1 year preceding the initiation of crizanlizumab treatment and extended through 1 year of therapy. Eligible patients were identified from the Novartis local cohort, classified as Expanded Access Program (EAP). Demographic information, VOC events, and clinical and follow‐up variables were collected. Results Among the 81 patients with at least one VOC prior to crizanlizumab initiation, 61 patients experienced VOC during the posttreatment period. Despite this reduction in the number of patients with VOCs, event‐based analyses showed a decrease in the number of VOCs requiring hospitalization (from 105 to 74) and emergency care (from 213 to 82) after crizanlizumab initiation. The median time between first and second VOCs requiring a healthcare visit increased from 1.66 to 1.84 months, and the median time between VOCs requiring hospitalization increased from 2.22 to 3.73. Conclusions These results suggest that treatment with crizanlizumab is associated with a reduction in VOC occurrence in all genotypes, SCD general complications, duration of hospitalization (in VOCs that required emergency care), and symptoms during VOCs.
Transfusion-dependent thalassemia (TDT) is characterized by chronic hemolysis, ineffective erythropoiesis, and iron overload, resulting in persistent oxidative stress and systemic inflammation. Patients with TDT are also at increased risk of thromboembolic complications, suggesting disturbance of hemostatic and immune-related pathways. However, the molecular links between hemolysis, complement-associated pathways, and coagulation abnormalities remain incompletely defined. In this study, quantitative serum proteomics was used to identify differentially expressed proteins in patients with TDT compared with healthy controls. Pathway enrichment and network analyses demonstrated coordinated alterations in proteins associated with complement regulation, coagulation pathways, lipid transport, and regulatory mechanisms. Complement component C3 and prothrombin showed increased abundance, whereas several regulatory and heme-scavenging proteins, including alpha-2-macroglobulin, hemopexin, and ceruloplasmin, were reduced in TDT patients. Selected proteins were further evaluated using ELISA validation, which supported the direction of the proteomic findings. Overall, these results identify a serum proteomic profile consistent with perturbation of complement and coagulation-related pathways in TDT in the context of chronic hemolysis and oxidative stress. This system-level view provides additional insight into thromboinflammatory mechanisms in TDT and supports further investigation of these pathways as potential biomarkers of disease severity.
Advances in hematopoietic stem cell transplantation (HSCT) for adolescents and adults with sickle cell disease (SCD) using non-myeloablative (NMA) regimens have demonstrated promising outcomes in terms of mortality and reduction in disease/transplant-related complications. Given these outcomes, assessing HSCT’s impact on healthcare use and productivity is key to guiding cost-effectiveness decisions and informed treatment choices. In the current study, we compare healthcare utilization (HCU) and productivity loss in patients with SCD who have undergone HSCT to HSCT-eligible patients but not transplanted yet. This was a cross-sectional study. Participants reported the number of HCU events in the preceding 6 months in addition to their sociodemographic information. Generalized linear regression models were used to estimate the differences between the two groups. A total of 181 SCD patients aged 14 years or older (108 recipients and 73 candidates) completed a one-time online survey. All measured HCU events (ED visits, blood transfusion, and hospitalization) were significantly lower among recipients compared to candidates (p < 0.001). Likewise, recipients, compared to candidates, demonstrated significantly better Work Productivity and Activity Impairment (WPAI) scores (p < 0.001). In conclusion, recipients of NMA HSCT reported significantly lower HCU and better productivity as compared to candidates. These findings indicate that NMA HSCT exhibited a positive impact on the socioeconomic aspects of their life and provide implications into health policy determinations, decision makers, and healthcare providers for the optimal use of the NMA HSCT in adolescents and adults with SCD.
Introduction:Thrombotic risk refers to the likelihood of a thrombus, or blood clot, forming in the vascular system, which can obstruct blood flow and cause serious complications. This risk is closely linked to the function of physiological coagulation inhibitors. The causes of thrombosis are multiple, including damage to the vascular wall caused by blood vessel injury, trauma, surgery, or vascular inflammation; these can initiate stasis, or slow blood flow, thus promoting blood cell aggregation and clot formation. These factors are encountered during several pathological processes, including sickle cell disease. This study aimed to evaluate physiological coagulation inhibitors and their association with inflammation and to deduce the thrombotic risk in patients with sickle cell disease. Materials and methods:We conducted an analytical cross-sectional study over 1 year. The Bafoussam Regional Hospital served as the framework for this research. The study population consisted of homozygous sickle cell patients regularly followed in the hospital and recruited in the hematology department. The collected blood samples were sent to the Hematology and Hemostasis laboratory. The analyses carried out were the hemogram by the flow cytometry method; the determination of the activity of protein C, protein S, and antithrombin with a coagulometer by the magnetic method; and finally, the exploration of inflammation from CRP dosage by the ultrasensitive method and the dosage of interleukin-6 by the ELISA sandwich method. The data obtained were recorded in an Excel 2013 spreadsheet and analyzed using the statistical software R Version 4.1.1. Results:A total of 150 homozygous sickle cell patients were included in this study. Of these, 98 (65.34%) presented a thrombotic risk. A high frequency of anemia (94.7%) was reported. Moreover, 82.7% and 64.0%, respectively, of leukocytosis and thrombocytosis were reported. An elevation of inflammatory parameters was observed characterized by an elevation of CRP (100% of patients at risk versus none in the group without it) and a significantly higher median IL-6 level in the group of patients at thrombotic risk (50.1 [28.7-76.6] [pg/mL]) compared to those without it (32.3 [14.1-44.4] pg/mL) (p = 0.001). A decrease in coagulation inhibitors was observed, notably a significant decrease in protein C in patients with thrombotic risk compared to those without, with respective proportions of 71.6% and 50% (p = 0.001). The median antithrombin level was 79.0% (77.0-92.0) in patients with thrombotic risk and 94.0% (84.0-102) in those without (p < 0.001). Patients at risk of thrombosis had a significant decrease in protein S (76.6% vs. 50% [p < 0.001]); the decrease in the latter was identified as a predictive factor of thrombotic risk during sickle cell disease (aOR = 1.5 [1.1; 5.2]; p = 0.04). Conclusion:This study reports a significant frequency of thrombotic risk in the sickle cell population characterized by a decrease in physiological coagulation inhibitors. This highlights the need for a systematic exploration of these markers for the prevention of cardiovascular disease in this patient group.
Objective To evaluate the effectiveness and safety of anagrelide in Colombian patients with essential thrombocythemia (ET) treated under routine clinical practice conditions. Materials and Methods A prospective, multicenter, real‐world observational study was conducted across 15 departments in Colombia. Adult patients diagnosed with ET were included. Patients initiated treatment with anagrelide and were followed for 12 months. The primary outcome was the change in platelet count. Secondary outcomes included changes in hemoglobin, leukocyte counts, and reporting of adverse events. Response was classified as complete (< 400,000/μL), partial (400,000–600,000/μL), or absent (> 600,000/μL), adapted from ELN/IWG‐MRT criteria. Results Of the 103 patients recruited, 53 met the inclusion criteria. The mean age was 62.2 years, and 69.8% were female. Anagrelide was used as second‐line therapy in 88.7% of patients. At 12 months, 22.0% achieved complete response (< 400,000/μL) and 9.8% partial response (400,000–600,000/μL). Complete responses were more frequent in patients with dose escalation (38.2% vs. 9.5%; p = 0.0008). A progressive reduction in platelets was observed, particularly significant in those with baseline thrombocytosis > 600,000/μL ( p < 0.001). No significant changes were identified in hemoglobin or leukocytes. Most adverse events were mild and consistent with the known profile of the drug: headache (39.6%), gastrointestinal symptoms (37.7%), and palpitations (26.4%). No serious treatment‐related events were reported. Conclusions In real‐world settings, anagrelide was effective and safe for the treatment of ET, yielding sustained platelet reduction and a favorable tolerability profile. Active dose titration was associated with higher response rates, underscoring the need for close monitoring in clinical practice.
AL amyloidosis often presents with nonspecific symptoms, leading to delayed diagnosis and increased early mortality, even in patients with known multiple myeloma or other monoclonal gammopathies. This study assessed the cardiac amyloid artificial intelligence electrocardiogram (CA-AI-ECG) tool’s utility in predicting treatment outcomes and detecting CA in a cohort of multiple myeloma patients undergoing autologous stem cell transplantation (ASCT). Among 2934 multiple myeloma patients studied, 81% received early ASCT (< 12 months from diagnosis) and therefore had an early ECG performed (early ASCT/ECG cohort), with 5% having a positive pre-ASCT CA-AI-ECG score. In the early ASCT/ECG group, a positive CA-AI-ECG score did not correlate with decreased overall survival. However, in the delayed ASCT/ECG group, a positive CA-AI-ECG score was associated with significantly lower overall survival (HR: 1.73, 95% CI: 1.07–2.78), remaining an independent risk factor on multivariate analysis. The observed overall survival difference between cohorts may be attributable to the longer time interval from diagnosis to ECG in the delayed ASCT group, leading to prolonged light chain exposure. During follow-up, 25 patients developed AL amyloidosis, of which, 12 patients had systemic involvement. Those with a positive pre-ASCT CA-AI-ECG were five times as likely to progress to systemic amyloidosis compared to those with a negative score (LR 5.0 p=0.025). These findings suggest that the CA-AI-ECG tool may enhance subclinical CA detection, especially as an early screening tool.
Spontaneous tumor lysis syndrome (sTLS) is a life-threatening oncometabolic emergency that remains poorly documented in low-resource settings. This retrospective cohort study aimed to assess the prevalence, risk factors, and prognostic implications of sTLS in adults with aggressive hematologic malignancies in Dakar, Senegal. Twenty-six newly diagnosed patients (18 with aggressive non-Hodgkin lymphomas and 8 with acute leukemias) were included between April 2020 and April 2021. sTLS was defined according to the Cairo–Bishop criteria. Clinical, biological, and survival data were analyzed using univariate tests and Kaplan–Meier estimates. The prevalence of sTLS was 30.8% (8/26). Acute kidney injury was the only factor significantly associated with sTLS (p=0.005), while hyperleukocytosis and bulky tumor burden were not correlated. All patients received hyperhydration, and patients at high risk received allopurinol, resulting in normalization of metabolic disturbances within 1 week. Early mortality (≤ 3 months) occurred in 15.4% of patients, and overall mortality reached 38.5%, mainly due to advanced disease. Survival did not differ significantly between patients with and without sTLS (log-rank = 0.7). In conclusion, sTLS is frequent in Senegalese patients with aggressive hematologic malignancies. Early detection and basic supportive care remain effective for preventing fatal complications in the absence of rasburicase.
Haematopoietic stem cell transplantation (HSCT) is an essential method used in the treatment of several haematological, immunological and metabolic disorders in both humans and animal patients. It serves as curative method for both benign and malignant haematological conditions. Blood cancers are haematological malignancies or liquid tumour that affect the lymph nodes, bone marrow and blood. Complex mechanisms, such as angiogenesis and the activation of new blood vessel development by cancer cells through factors, such as VEGF and bFGF, which promote tumour growth, metastasis and proliferation, are involved in the progression of blood malignancies. The therapeutic application of HSCT in congenital immunological deficiencies, diseases of bone marrow failure and malignant haematological disorders demonstrate the therapeutic efficacy. The therapeutic potential of HSCs lies in their self-renewal capacity and their ability to differentiate into myeloid and lymphoid lineages, pinpointing their essential for maintaining haematopoiesis. A variety of sources can provide haematopoietic stem cells, which are necessary for the synthesis of different types of blood cells. As an alternative to bone marrow and peripheral blood stem cells, umbilical cord blood (UCB) has become the most common valuable source of haematopoietic stem cells. Information sorted from PubMed, Google Scholar, Elsevier, Taylor & Francis, MDPI, SCIENCEDOMAIN, ScienceDirect and other related sources was reviewed. In this review, appreciable information on the roles of HSCT in the management of blood cancers was highlighted.
Introduction:The preanalytical phase of blood sample collection, storage, and transportation is crucial in clinical laboratory practice. This study examined the impact of preanalytical storage time and the presence of air in the test tube on viscoelastic and traditional coagulation tests and platelet activation markers. Methods:Blood samples from ten healthy donors were divided into Vacuette sodium citrate and K2E EDTA tubes, with and without air, and stored at room temperature with gentle agitation for up to 48 h. Coagulation tests (ROTEM, APTT, INR, and PTF1 + 2), platelet activation markers (β-thromboglobulin and sP-selectin), blood gases and metabolites (pH, pCO2, pO2, bicarbonate, hemoglobin, sO2, K+, Ca2+, glucose, and lactate), and hemolysis (free hemoglobin and absorbance) were measured. Results:In citrated blood samples, ROTEM parameters, APTT, and PTF1 + 2 remained stable during 6 hours of storage at room temperature, and INR was stable for 48 h. In EDTA blood, β-thromboglobulin increased initially and then remained stable for 6 hours. sP-selectin remained stable for 6 hours. In EDTA blood samples, hemolysis remained minimal during storage, but cell death revealed by a lactate dehydrogenase increase was observed after 24 h. Blood gases and metabolites exhibited rapid changes, underscoring the need for immediate analysis. No significant differences were observed between samples with and without air in the test tubes. Conclusion:ROTEM, APTT, PTF1 + 2, and platelet activation markers were stable for 6 h and INR was stable for 48 h. Removing ambient air from blood samples did not extend preanalytical stability. These insights are valuable for optimizing preanalytical practices in clinical laboratories, potentially improving patient care and reducing costs.
On‐demand clotting factor concentrate (CFC) replacement therapy remains the predominant management strategy for adult people with hemophilia in Indonesia due to resource and policy limitations. However, this approach does not prevent long‐term complications. This study aimed to describe the bleeding profile, complications, and invasive procedures among adult people with hemophilia A and B receiving on‐demand CFC therapy in a tertiary referral hospital. We conducted a retrospective cross‐sectional study at Cipto Mangunkusumo Hospital, Jakarta, from March 2024 to May 2025. Adult patients (≥ 18 years) diagnosed with hemophilia A or B were consecutively recruited through nonprobability sampling. Demographics, comorbidities, bleeding manifestations, CFC treatment patterns, complications, and history of invasive procedures were collected from electronic medical records and analyzed descriptively. A total of 120 patients were included (94 with hemophilia A and 26 with hemophilia B), with most having severe disease (80%). Comorbidities were present in 43.3%, predominantly hepatitis C (21.7%). Patients averaged three outpatient visits per month, receiving 24.5 IU/kg of factor VIII or 17.6 IU/kg of factor IX per infusion. The median annualized bleeding rate was 26 for hemophilia A and 31 for hemophilia B. All patients experienced hemarthrosis, commonly affecting multiple joints, especially the knees (65.8%), elbows (41.7%), and ankles (40.9%). Other frequent bleeding manifestations included gum bleeding (19.2%) and soft tissue hematomas (15.8%). Hemophilic arthropathy was observed in 10.9%, primarily involving the knees, while hemophilic pseudotumor occurred in 1.7%. Invasive procedures were recorded in 26.5% of patients, more frequently among those with hemophilia A compared with hemophilia B (28.9% vs. 19.1%). The most common procedures were odontectomy and orthopedic surgeries. Episodic on‐demand CFC replacement therapy remains the primary treatment for adult people with hemophilia in Indonesia. Bleeding manifestations remain frequent, and a substantial proportion of patients have undergone invasive procedures. These findings support the need to prioritize prophylactic therapy and early management of hemophilia‐related complications.
Imatinib mesylate is a small molecule inhibitor targeting the BCR‐ABL tyrosine kinase. However, some CML patients develop resistance to imatinib. This resistance is commonly associated with mutations in the kinase domain of BCR‐ABL, notably the prevalent T315I and E255K mutations. We evaluated the frequency of E255K and T315I mutations in CML patients who do not respond to imatinib therapy. Twenty imatinib‐resistant CML patients were selected from a total cohort of 100 CML patients undergoing regular follow‐up. Genomic screening for the T315I and E255K mutations was carried out using a two‐step molecular approach: initial reverse transcription‐PCR (RT‐PCR) amplification of the ABL kinase domain, followed by restriction fragment length polymorphism (RFLP) analysis. The subsequent clinical management and outcomes for patients harboring these mutations were meticulously tracked and analyzed. The T315I and E255K mutations were present in 20% (4/20) and 10% (2/20) of the imatinib‐resistant patients, respectively. All patients required an immediate switch to second‐line tyrosine kinase inhibitors. Notably, one patient with the T315I mutation secured ponatinib and achieved remission, while three other patients underwent allogeneic stem cell transplantation. Two patients carrying the E255K mutation ultimately progressed to blast crisis and died, highlighting the particularly dire prognosis associated with this mutation. The T315I and E255K mutations are significant contributors to imatinib resistance in our patient population and are linked to an aggressive clinical course. These findings underscore the critical importance of integrating routine mutation screening into clinical practice to enable early treatment modification, facilitate access to advanced therapies, and ultimately improve patient outcomes.
Patients with sickle cell disease (SCD) experience high rates of emergency department (ED) visits and hospitalizations, primarily owing to vaso-occlusive crises (VOC). In Edmonton, patients followed by the Centre for Bleeding and Rare Blood Disorders (CFBD) have access to the Medical Outpatient Unit (MOU) to receive care from a personalized pain plan for their VOC episodes as an alternative to ED presentation. In this study, we performed a retrospective analysis to evaluate the use of the MOU, thereby diverting patients from presenting to acute care. A chart review was completed of patients 18 years or older with SCD who utilized the MOU from November 2019 to December 2022. Any repeat encounters within 7 days were assumed to be part of the same VOC event. Twenty-six patients with SCD presented to MOU during the time of this study. There was a total of 136 encounters (median 2, maximum 25) accounting for 96 separate episodes of VOC (median 2, maximum 19). The most common interventions included administration of IV fluids in 129 encounters (95%) and use of pain medications in 64 encounters (47%). Patients only required assessment by nursing in the majority (80%) of encounters and were discharged home safely from MOU in 99% of encounters, with only two encounters resulting in patients requiring assessment in ED. Seventy of 96 episodes of VOC treated in MOU did not have an associated presentation to ED within 30 days (73%). The total ED presentations of this cohort decreased from 59 in 2016–2018 to 38 during this study. The use of the MOU was effective strategy for providing outpatient assessment and treatment of VOC, and there was an associated decrease in ED visits in this cohort.
Objective Treating Diffuse Large B-Cell Lymphoma (DLBCL) in elderly patients is challenging. There is limited data available from Low- and Middle-Income Countries (LMICs) on elderly DLBCL. We analyzed the presentations and survival outcomes of patients with DLBCL according to their socioeconomic status. Methodology This was a multicenter retrospective study conducted from 2015 to 2023. We included 176 patients aged 60 years or older. The variables examined were age, gender, subtype, resource environment and treatment received. Kaplan-Meier curve was created for the entire patient cohort; t-test was utilized to compare means, Disease-Free Survival (DFS) and Overall Survival (OS), with a significance level of p < 0.05. Analysis was performed using SPSS version 29. Results The median age was 66 years (range: 60–89 years). Ninety-three (57%) patients were treated in limited resource settings, while 43% had enhanced resources. ECOG performance scores between 2 and 3 were present in 71%. Median IPI score was 3. RCHOP regimen was administered to 51% (n = 81) patients, and CHOP regimen to 20% (n = 32) patients. In 21% (n = 38) salvage treatment was given due to relapsed/refractory disease. None of the patients in this group received consolidation with autologous stem cell transplant. The entire cohort’s OS was 12-months, while DFS was 8-months. OS (33.9% vs. 8.2%; p = 0.00) and DFS (29% vs. 5.9%; p = 0.00) were better in patients with enhanced resources. The median DFS of patients treated in enhanced settings was 1.3-years versus 0.4-years in limited resource settings (p < 0.0001) Conclusion Survival rates were lower for patients receiving treatment in resource-limited settings. Outcomes can be improved with early referral and inclusion of Rituximab. Enhanced geriatric assessments along with better supportive care is essential.
Patients with multiple myeloma (MM) and secondary immunodeficiency (SID) are at risk of infection-related morbidity and mortality owing to the disease process and treatment toxicity. This retrospective cohort study used anonymized data from the Optum-Humedica database (October 2015-March 2020) to assess the burden of infection in patients with MM and SID versus patients without SID. Patients aged ≥ 18 years with confirmed MM were assigned to SID and no-SID cohorts using an algorithm considering serum IgG levels < 5.0 g/L, hypogammaglobulinemia diagnosis codes, and ≥ 1 major infection. Patients with SID were stratified into those treated or not treated with immunoglobulin replacement therapy (IgRT and no-IgRT cohorts). At 12 months follow-up, a greater proportion of the SID cohort than the no-SID cohort experienced infections (58.9% vs. 31.3%; p < 0.001), severe infections (29.8% vs. 10.6%, p < 0.001), and infection-related hospitalizations (26.9% vs. 9.1%; p < 0.001). Bacterial infections were the most common infection type (SID cohort, 49.2%; no-SID cohort, 26.1%; IgRT cohort, 70.4%; no-IgRT cohort, 45.1%). Use of anti-infectives and healthcare resource utilization was higher in the SID cohort than in the no-SID cohort. Median overall survival was shorter in the SID versus no-SID cohort (12.6 vs. 14.7 months; p < 0.001) and was similar in the IgRT versus no-IgRT cohort (8.5 vs. 12.5 months; p = 0.446). Among patients with MM and SID, the higher infection burden in patients treated with IgRT than no-IgRT suggests the IgRT cohort was a more vulnerable population. A better understanding of SID burden may improve outcomes for patients with MM.
Background:Primary warm autoimmune hemolytic anemia (PWAIHA) is a subtype of autoimmune hemolytic anemia (AIHA) characterized by premature destruction of red blood cells (RBCs) due to autoantibodies, typically immunoglobulin G (IgG), the cornerstone of which is a positive direct antiglobulin test. The indirect antiglobulin test (IAT) is used as a screening test to detect common and clinically significant RBC alloantibodies in patient serum. Objective:This study aimed to analyze the prevalence, clinical characteristics, and impact of positive IAT in adult patients with PWAIHA at the time of diagnosis, as well as the response to corticosteroids as the first-line treatment. Methods:This single-center retrospective case-control study was conducted between September 2002 and May 2024 at King Abdullah University Hospital. We analyzed data from 80 adult patients diagnosed with PWAIHA, aged a minimum of 16 years. After recording baseline investigations, including IAT and antinuclear antibody (ANA), all patients were treated with corticosteroids as first-line treatment. The response rate and prevalence of ANA were compared between the IAT-positive and -negative PWAIHA groups. Results:Baseline IAT positivity was found in 65% of our patients. Both groups were comparable in terms of age, sex, and hemoglobin levels. Serum LDH presentations were higher in positive IAT patients, response to corticosteroids was numerically higher in patients with negative IAT (54%) than those with positive IAT (33%), and after tapering steroids, patients with a positive IAT had higher rates of relapses compared with those with negative IAT. Positive ANA was found only in IAT-positive patients (25%), which was statistically significant. Conclusion:An association between baseline IAT positivity and lower rates of complete response was observed in PWAIHA patients. The presence of IAT serves as a prognostic indicator and aids in the decision-making process regarding treatment options. Furthermore, positive IAT results are linked to a higher prevalence of ANA positivity.
Background:The Hyper-CVAD protocol is a chemotherapy regimen widely used in hematological malignancies. It is considered one of most promising remission-leading treatment option for adults with acute lymphoblastic leukemia (ALL). Objective:To determine the outcomes of adults with ALL treated with the Hyper-CVAD protocol in Ecuador. Methods:This multicenter, retrospective, cross-sectional study compared the outcomes of ALL patients aged 15 years and older treated with hyper-CVAD protocol in 8 specialized centers. Results:139 patients with ALL treated with the Hyper-CVAD protocol were included. A total of 79 were female (56.8%) and the majority had a B-type phenotype 130 (93.5%). A complete response (CR) was achieved in 64.3%. Relapse was confirmed in 52.7% of those who obtained CR. Negative minimal residual disease (MRD) was achieved in 20% of patients. The median overall survival (OS) was 11 months (95% CI: 7.49-14.50) with a 5-year OS of 14.0%. A total of 12 (8.6%) deceased during induction phase. Conclusion:Adult patients treated with the Hyper-CVAD protocol in Ecuador achieve rates of CR, MRD, and OS lower than those presented in international cohorts, as well as higher rates of treatment-related toxicity.
