Mediterranean Journal of Hematology and Infectious Diseases最新文献

Background: Hematopoietic stem transplantation (HSCT) from matched related donors (MRD) is offered as a curative therapeutic option in children with Sickle cell disease (SCD).

Objective: We wanted to assess the outcome and long-term complications observed in children undergoing HSCT at a single transplant center in Saudi Arabia.

Patients and methods: One hundred and twenty-nine children were transplanted for severe Sickle cell disease (SCD) consecutively from 2006 to 2020 at our center. The main transplant indication was cerebral vasculopathy in 57 (43%), followed by the recurrent vaso-occlusive crisis (VOC) in 47 (36%). Median age at transplant was 9.1 years (range, 1.5-13.9 years). All patients received myeloablative conditioning with Busulfan, Cyclophosphamide, and Anti T-Lymphocyte Globulin (Grafalon®): BU/CY/ATG in 114 (88.4%), BU/CY in 13 (10%) and other in 2 (2%). Bone marrow was the main stem cell source in 123 (95%).

Results: All patients showed granulocyte engraftment. Acute graft-versus-host-disease (aGVHD) and chronic GVHD were observed in 26 (20%) and 12 (9%) patients, respectively. At a median follow-up of 4.36 years (range, 0.13-15.5 years), 10-year overall survival (OS) and event-free survival (EFS) of 94% and 91% was observed. The OS and EFS were significantly better in patients receiving BU/CY/ATG when compared to BU/CY (OS: 97.4%±1.5%, vs. 76.2%±12.1 P=0.003 and EFS: 94.7%±2.1% vs. 76.2%±12.1%, P=0.019).

Conclusion: HSCT for children with sickle cell disease from fully matched siblings offers the best outcome using myeloablative conditioning. However, significant toxicities were observed secondary to myeloablative regimens, in particular long-term complications, which demands exploring the use of less toxic regimens.

Heparin-binding protein (HBP) is a granule protein derived from neutrophils, located in secretory vesicles and neutrophilic granules, also known as cationic antimicrobial protein of 37 kDa (CAP37) or azurocidin. This study evaluates the diagnostic and prognostic value of HBP levels in relation to infection, organ dysfunction, and mortality in adult patients. A systematic review and meta-analysis were conducted by searching PubMed, Web of Science, EMBASE, and the Cochrane Database from their inception through June 2024. Original studies assessing HBP levels' diagnostic and prognostic utility in predicting infection and disease severity in critically ill adult patients were included. The primary outcome was the diagnostic and predictive role of HBP in infection and severity. The Quality Assessment of Diagnostic Accuracy Studies 2 (QUADAS-2) tool was used to evaluate bias risk. A total of 56 studies involving 11,486 patients were included. Pooled analysis showed HBP had a sensitivity of 0.87 (95% CI, 0.82-0.91), specificity of 0.87 (95% CI, 0.79-0.92), and an AUC of 0.93 (95% CI, 0.91-0.95) for infection diagnosis. For prognostic assessment, sensitivity was 0.77 (95% CI, 0.74-0.80), specificity was 0.72 (95% CI, 0.68-0.76), and AUC was 0.81 (95% CI, 0.78-0.85). HBP outperformed procalcitonin (PCT), C-reactive protein (CRP), and white blood cell count (WBC) in diagnosing and predicting critical illness. No publication bias was detected. HBP demonstrates high sensitivity and specificity for diagnosing infections in critically ill adult patients. Additionally, it effectively predicts disease progression, including organ dysfunction and mortality, surpassing traditional biomarkers such as PCT, CRP, and WBC. All that cannot be true for subjects with severe neutropenia.

Chimeric antigen receptor (CAR) T-cell therapy has improved the outcomes of patients with relapsed/refractory B-cell lymphomas, B-cell acute lymphoblastic leukemia, and multiple myeloma. However, CAR-T cell therapy is also associated with distinct toxicities that contribute to morbidity and mortality. A large number of studies now define the different toxicities associated with CAR-T cell therapy and have, in part, clarified their mechanisms. In particular, cytokine release syndrome (CRS) and immune effector cell-associated neurotoxicity syndrome (ICANS) are the two main acute toxicity events that occur after CAR-T cell infusion. Other CAR-T-related toxicities occur later after CAR-T cell infusion and include B-cell aplasia, hypogammaglobulinemia, infections, and cytopenias. Infections represent the main cause of non-relapse death observed in patients undergoing CAR-T cell therapy. Second primary malignancies are rare and are mainly represented by myeloid malignancies.

Background: To investigate the occurrence of Plasmodium vivax infections in Duffy-negative individuals, challenging the long-held belief that P. vivax requires the Duffy antigen receptor for chemokines to infect human erythrocytes.

Materials and methods: In the present study, 365 samples were screened using serological techniques, PCR-RFLP analysis, and DNA sequencing of the ACKR1 gene promoter region mutation to identify Duffy-negative individuals. P. vivax infection was detected using PCR targeting the 18S rRNA gene and microscopic examination of Giemsa-stained blood smears.

Results: Five individuals (1.36%) were confirmed Duffy-negative (Fy^(a-b-)). Surprisingly, 3 out of these 5 Duffy-negative subjects (60%) were infected with P. vivax, as confirmed by both microscopy and PCR. Various parasite stages were observed in infected Duffy-negative samples, with parasitaemia ranging from 0.01% to 0.5%.

Discussion: Our findings provide compelling evidence that P. vivax can infect Duffy-negative individuals, suggesting the existence of alternative invasion pathways or adaptations. This has profound implications for P. vivax biology, evolution, and global distribution. The burden of vivax malaria may be underestimated, particularly in regions with a high prevalence of Duffy negativity. This study highlights the need to reevaluate P. vivax epidemiology, diagnostic approaches, and control strategies, especially in areas previously considered at low risk. Further research is needed to elucidate the mechanisms enabling P. vivax invasion of Duffy-negative erythrocytes and to assess the clinical and epidemiological consequences of these infections.