Journal of Blood Medicine最新文献

Purpose: Immune thrombocytopenia (ITP) is an autoimmune disorder characterized by bleeding, yet paradoxically, it can also predispose patients to thrombotic events; therefore, investigating high-risk factors for thrombosis in ITP patients and developing a predictive model is essential.

Patients and methods: A total of 1112 patients were diagnosed with ITP between January 2018 and December 2023. Excluding 216 patients under the age of 18, a total of 896 adult ITP patients were included in this study (of whom 101 developed thrombosis). Patients were randomly allocated to the training set (n=628) and validation set (n=268) in a 7:3 ratio.

Results: Atrial fibrillation, peripheral vascular disease, venous thromboembolism history, pneumonia < 1 month, cerebrovascular events history, and D-dimer levels were identified as predictive factors for thrombosis in ITP patients. The six factors formed 57 unique combinations, providing robust predictive power for thrombosis in ITP under different clinical scenarios. In the training set, the area under the curve (AUC) for the nomogram was 0.656 (95% CI: 0.578-0.735) to 0.931 (95% CI: 0.902-0.960) and the AUC in the validation set was 0.539 (95% CI: 0.425-0.653) to 0.893 (95% CI: 0.828-0.957). The calibration curve demonstrated good concordance between the model's predicted probabilities and actual observed probabilities, and the decision curve analysis indicated that the model had significant clinical utility.

Conclusion: This cohort study developed a simple and practical predictive model (ITP-THROMBO) for estimating thrombosis risk in ITP patients; this model facilitates rapid identification of ITP patients at high thrombotic risk, enabling timely decision-making support for personalized treatment planning.

Febrile neutropenia develops in approximately three-quarters of recipients of allogeneic hematopoietic stem cell transplantation (allo-HSCT). Among them, carbapenem-resistant Enterobacterales (CRE) associated mortality reaches up to 70%. Thus, rapid identification of CRE-high-risk patients and prompt empiric CRE coverage are critical. Two allo-HSCT recipients whose febrile neutropenia persisted on empiric carbapenem were promptly escalated to imipenem/cilastatin/relebactam, alone or combined with aztreonam and tigecycline-before culture confirmation. Defervescence occurred within 24-48 h, inflammatory markers declined progressively, and clinical infection control was achieved. Successful neutrophil and platelet engraftment followed. Immediate empiric imipenem/cilastatin/relebactam for CRE-high-risk allo-HSCT recipients with febrile neutropenia rapidly controlled infections and permitted successful hematopoietic recovery. Prospective studies are warranted.

Background: Blood transfusion during hip fracture surgery can significantly influence patient outcomes. This study aimed to identify risk factors for post-operative blood transfusions and their impact on clinical outcomes, including all-cause mortality.

Methods: A post-hoc analysis was conducted on data from a multicentric observational study in Jordan. Demographics, preoperative variables, intraoperative details, and postoperative outcomes were analyzed. Logistic regression identified risk factors for transfusions, and Cox proportional hazards models assessed associations with mortality.

Results: The study included 1040 patients who underwent hip fracture repair (35.87% received transfusion and 64.13% did not receive transfusion). Patients who received transfusion were older (median age 79 vs 77 years, p=0.0015), more frequently females (60.59%), and had lower preoperative hemoglobin levels (10.85±1.75 vs 12.62±1.61 g/dL, p<0.001). Clopidogrel use (10.99% vs 6%, p=0.004), unstable intertrochanteric fractures (53.08% vs 42.13%, p=0.001), and ICU admissions (19.03% vs 6.45%; p<0.001) were significantly more common in patients who received transfusion. Transfused patients experienced longer hospital stays (median 7 [IQR=5-10] vs 6 [IQR=4-8] days, p<0.001), higher readmission rates (13.4% vs 8.85%; p=0.021), and increased all-cause mortality (18.23% vs 11.24%; p=0.002). However, this observed increase in mortality did not remain significant after multivariate adjustment. At multivariate analysis, blood transfusion was independently associated with ICU admission (OR=2.17, 95% CI=1.28-3.66,p=0.004), and longer hospital stay (OR=1.05, 95% CI=1.02-1.09, p=0.005), while no independent association between blood transfusion and all-cause mortality was found (HR=1.15, 95% CI=0.79-1.66, p=0.465).

Conclusion: Blood transfusion in hip fracture surgery are linked to longer hospital stay and ICU admission, but not to increased all-cause mortality. Careful management of hemoglobin levels and transfusion practices is crucial.

Background: Incompatible cross-matching at room temperature can delay critical blood transfusions. We report that red cells from patients requiring blood transfusion agglutinated after cross-matching at room temperature, but changed from agglutination to dispersion after a water bath at 37°C. The aim was to investigate whether re-cross-matching at 37°C is necessary for some patients with incompatible cross-matching.

Methods: This prospective observational study analysed all incompatible cross-matched blood samples at the Blood Transfusion Department of Chifeng Municipal Hospital from 1 May 2024 to 31 July 2024. Cross-matching was performed using a fully automated system and the Polybrene method. For incompatible samples, the Polybrene method was repeated after the samples were placed in a 37°C water bath for 5 minutes.

Results: Thirty patients with cross-match incompatibilities were included. Among these, agglutination was reversed after the 37°C water bath in 28 patients, indicating compatibility. Only 2 patients had true blood group incompatibility. We detail the results of one representative patient from the 28 with reversed agglutination. This patient showed agglutination using the fully automated device and the Polybrene method, but not with the saline method. After a 37°C water bath, the agglutinated erythrocytes dispersed into a single-cell state upon microscopic examination.

Conclusion: For selected patients, cross-matching at 37°C can resolve false-positive agglutination, ensuring transfusion safety and timely access to blood products. This simple method can be integrated into the clinical cross-matching workflow.

Purpose: All umbilical cord blood (UCB) used for hematopoietic cell transplantation (HCT) must pass through a series of strict tests, including volume, total nucleated cell count, microbial contamination, hematopoietic stem cells content, and activity. A strictly standardizing collection procedures for UCB within hospitals is a critical step in preserving hematopoietic stem cells resources.

Methods: The study included a total of 560 participants, with 337 in the control group and 223 in the observation group, collected from January 2022 to December 2024. Participants were randomly assigned to two groups. The UCB collected from newborns before the initiation of Quality Control Circle (QCC) activities served as the control group, whereas those collected after the initiation of QCC activities formed the observation group. Through the establishment of QCC nursing management, the reasons for UCB storage failures within the department were analyzed, and corresponding countermeasures were formulated. Statistical analyses were performed to compare the reasons for failure of UCB storage, success rate, and quality indicators of successfully stored UCB between the two groups.

Results: There were no statistically significant differences in the general clinical data of the newborns between the two groups. After the implementation of QCC activities, the rate of successfully stored UCB samples in the observation group was 90.6%, which was significantly higher than that of 84.3% in the control group (P=0.031). There were no statistically significant differences in the quality indicators of successfully stored UCB between the two groups.

Conclusion: The implementation of QCC in UCB collection increased the success rate by 6.3 percentage points without compromising cellular quality, supporting its application as a continuous improvement strategy in obstetric settings.

Background: Iron deficiency anemia is a public health concern among children under five years old. This study aimed to determine the prevalence and determinants of iron deficiency anemia among children under five years attending Luwero General Hospital, Luwero District.

Patients and methods: This cross-sectional study included 380 children under five years old who attended Luweero Hospital. Participants were selected using a simple random sampling technique, and caregivers were interviewed using a structured questionnaire to collect demographic information. Laboratory tests performed were; hemoglobin and ferritin level, to evaluate anemia severity. Iron deficiency anemia was defined as serum ferritin levels below 12 μg/L. Association between determinants and iron deficiency was attained by developing bivariate and multivariate logistic regression models using STATA software version 14, and a p value of <0.05 was considered significant.

Results: This study found that anemia was prevalent amongst children under five years, affecting 38.4% (n = 146) of the sample, with iron deficiency anemia (IDA) identified in 16.6% (n = 63). Majority of the children with iron deficiency anemia had moderate anemia 32 (50.8%). At multivariate analysis, several factors were independently associated with iron deficiency anemia (IDA); male gender (aOR: 2.29, 95% CI: 1.20-4.35, p = 0.012), and children that had infection in the past 3 months (aOR: 5.62, 95% CI: 2.94-10.74, p < 0.000). However, parental age of 20-29 years (aOR: 0.21, 95% CI: 0.06-0.73, p = 0.014) and 30-39 years (aOR: 0.17; 95% CI: 0.05-0.59; p = 0.005) were independently associated with a significantly reduced odds of iron deficiency anemia (IDA).

Conclusion: The study highlights that iron deficiency anaemia is a significant health concern affecting 16.6% of the children studied. Male sex and a recent history of infection were found to be significant independent risk factors for IDA. Conversely, parental age between 20 and 39 years was identified as a strong independent protective factor, with the most pronounced reduction in IDA odds observed among children of parents aged 30-39. Anemia screening and intervention programs should be proactively targeted towards male children, especially those with a recent history of infection.

Erdheim-Chester Disease (ECD) is a rare histiocytic neoplasm characterized by multi-organ tissue infiltration by histiocytes. Clinical presentation and course can be heterogeneous, ranging from localized and asymptomatic bone lesions to a multisystem disease involving skin, cardiac, pulmonary, retroperitoneum, lymph node and central nervous system (CNS) with significant morbidity and mortality. Herein, we describe a rare case of a 61-year-old female patient with ECD with primarily brain and bone involvement who developed "progressive disease" in the CNS after 3 cycles of cladribine. The patient elected to forego any further treatment based on her clinical stability despite progressive disease imaging findings. Subsequent imaging while off treatment demonstrated marked interval improvement in the previously patchy areas of involvement and the patient has continued to have clinical stability 4 years after stopping therapy. The patient was deemed to have experienced pseudoprogression. Pseudoprogression of ECD has not been reported in the literature, and it may be associated with localized inflammatory cytokine release in response to treatment. The possibility of pseudoprogression needs to be considered in evaluating therapeutic response in ECD patients.

Paroxysmal Nocturnal Hemoglobinuria (PNH) is a rare hematologic disorder characterized by intravascular hemolysis through complement activation, bone marrow failure, and thrombosis. The advancement of complement biology has enabled better therapeutic approaches that lead to better clinical outcomes in patients with PNH and other complement-driven hemolytic disorders. The terminal complement inhibitor, eculizumab, was the initial drug available which significantly reduced hemolysis and thrombotic events but failed to resolve residual extravascular hemolysis and transfusion requirements. New therapeutic agents which target proximal complement factors C3, factor B and factor D demonstrate better control of intra- and extravascular hemolysis while decreasing transfusion requirements and improving patient quality of life. This review highlights the evolving therapeutic landscape in complement inhibition by summarizing clinical evidence for the terminal complement inhibitors, as well as pegcetacoplan, iptacopan, and danicopan as emerging agents for treatment of PNH and autoimmune hemolytic anemias-warm AIHA and cold agglutinin disease (CAD). The review also examines ongoing clinical trials and proposes future directions to optimize therapeutic outcomes to address remaining clinical challenges.

Background: Diffuse large B-cell lymphoma (DLBCL) is the most prevalent form of non-Hodgkin's lymphoma globally. SPAG5, a mitotic spindle protein, plays a significant role in DLBCL, where its abnormal expression is often associated with tumor growth, chemotherapy resistance, local recurrence, and poor prognosis.

Methods: A comprehensive analysis of SPAG5 expression across various cancer types was conducted using Timer 2.0 and Sanger Box 3.0. Subsequently, the expression levels of SPAG5 in DLBCL were investigated in comparison to normal samples. Receiver operating characteristic (ROC) curve was then generated to evaluate the diagnostic performance of SPAG5 for DLBCL. Furthermore, the functional role of SPAG5 was characterized, and its impact on the immune microenvironment of DLBCL patients was analyzed. Its potential in predicting immune checkpoint status and responses to immunotherapy was also evaluated.

Results: SPAG5 expression demonstrated significant heterogeneity across various cancer types, with a marked upregulation in DLBCL. The diagnostic efficacy of SPAG5 was moderate, yielding an area under curve (AUC) of 0.75. SPAG5 exerted a multifaceted influence on DLBCL progression by regulating critical cellular processes, including cell cycle dynamics, chromosomal segregation, and DNA homeostasis. Notably, patients with elevated SPAG5 expression had poorer survival outcomes than those with low expression. Analysis of the tumor immune microenvironment revealed a distinct pattern: high SPAG5 expression correlated with increased infiltration of resting natural killer (NK) cells, while being associated with reduced presence of regulatory T cells (Tregs) and follicular helper T cells (Tfh).

Conclusion: Our bioinformatics study elucidated the expression profile, diagnostic potential, and prognostic significance of SPAG5 in DLBCL, emphasizing the complex interplay between SPAG5 expression and the tumor immune landscape. Our findings suggested SPAG5 could be a candidate prognostic marker and potential therapeutic target for DLBCL.

Purpose: To investigate the distribution of antigen and allele frequencies of the MNS blood group system among multi-ethnic populations in East China, and to analyze the genetic polymorphism of uncommon phenotypes, thereby contributing to the enhancement of the regional blood type database.

Patients and methods: A total of 8606 whole blood samples were randomly collected from voluntary blood donors in East China between October 2023 and June 2024. MNS blood group phenotypes were identified using serological methods, and allele frequencies were analyzed and compared across populations. Genetic sequencing was performed on samples with uncommon MNS phenotypes.

Results: The study primarily included the Han, Hui, and Manchu populations. Among the Han population, the most prevalent phenotypes were M+N+S-s+ (45.21%), M-N+S-s+ (25.94%), and M+N-S-s+ (19.84%), respectively. Phenotypic distributions in most other ethnic groups were comparable to that of the Han population, except for the Yi population, which showed a significantly different distribution (P < 0.05). Furthermore, a rare serological phenotype, S-s-, was identified with a frequency of 0.01%. The allele frequencies of the MNS blood group system among different population in East China were consistent with the Hardy-Weinberg equilibrium (P > 0.05).

Conclusion: The MNS blood group system in East China's multi-ethnic populations exhibits polymorphism and regional specificity. Notable allele frequency differences exist between certain minority populations and the Han population. Therefore, it is essential to enhance the development of a regional blood type database tailored to East China in order to support precise clinical transfusion with robust data, including informed pre-transfusion antibody screening for high-risk groups.