Mediterranean Journal of Hematology and Infectious Diseases最新文献

Sickle cell disease (SCD) and transfusion-dependent β-thalassemia are no longer pediatric death sentences. With newborn screening, transfusions, and chelation therapy, patients now survive into their 4th-6th decade. Yet as they age, they face mounting complications -pain that never truly resolves, organs failing one by one, and profound isolation. Ironically, palliative care remains scarce despite the clinical complexity. This narrative review examines end-of-life care in these hemoglobinopathies, focusing on pain management, ethical tensions, and the psychosocial needs that intensify as death approaches. We reviewed literature from 2020 to 2025, international guidelines, and European frameworks. The evidence is clear: terminal SCD involves unpredictable crises and intractable pain; β-thalassemia brings slow cardiac decline and iron-laden organ failure. Both demand early palliative integration, yet both are drastically undertreated. Cultural beliefs heavily shape how families accept or reject end-of-life discussions. Disparities in opioid access, lack of disease-specific referral criteria, and absence of flexible hospice models create barriers that disproportionately harm marginalized patients. We conclude that hemoglobinopathy patients deserve the same anticipatory, culturally informed, multidisciplinary palliative care that we increasingly offer to cancer patients. Health systems must establish referral pathways specific to these diseases, permit palliative transfusions in hospice when appropriate, ensure equitable opioid access, and embed psychosocial support in hemoglobinopathy centers.

Background: Ubiquitination affects cancer progression by regulating both tumor-suppressing and tumor-promoting proteins in cancer. The current study sought to evaluate the role of TNF receptor-associated factor 6 (TRAF6) in the malignant proliferation of the human NK/T cell lymphoma cell line HANK1.

Methods: TRAF6 and MST1 expression levels in HANK1, KHYG-1, and SNK-6 cells were determined by RT-qPCR and Western blot analysis, followed by transfection of si-TRAF6 into HANK1 cells. Cell viability and proliferation were assessed by cell-counting kit-8 and 5-Ethynyl-2'-deoxyuridine (EdU) assays, and proliferating cell nuclear antigen (PCNA) expression levels in cells were determined by Western blot analysis. After that, cells were treated with MG132, followed by analysis of the binding of TRAF6 to macrophage stimulating 1 (MST1) via co-immunoprecipitation and the ubiquitination level of MST1 via the ubiquitination assay. The functional rescue experiment was performed with si-MST1 and si-TRAF6 in cells.

Results: TRAF6 was upregulated in HANK1 cells. Inhibition of TRAF6 reduced cell viability and the number of EdU-positive cells, and downregulated PCNA expression. TRAF6 binds to MST1 to promote ubiquitination-mediated degradation of MST1. After MG132 treatment, the ubiquitination level of MST1 declined. Silencing MST1 abolished the inhibition of TRAF6 on malignant proliferation of HANK1 cells.

Conclusion: TRAF6 was upregulated in HANK1 cells and bound to MST1 to promote ubiquitination-mediated degradation of MST1, consequently facilitating the malignant proliferation of HANK1 cells.

Thromboembolic and hemorrhagic complications are significant causes of morbidity and mortality in patients with acute leukemias (AL). While AL is characterized by a complex hemostatic imbalance, conventional coagulation tests and platelet counts offer limited predictive value for bleeding and thrombotic events. Global coagulation assays (GCAs), such as the Thrombin Generation Assay (TGA), provide a more comprehensive assessment of coagulation potential and may offer improved risk stratification. This prospective, single-center pilot study aimed to explore the utility of TGA in newly diagnosed adult patients with AL. Between February 2022 and September 2024, 111 patients were enrolled at the Department of Translational and Precision Medicine, Sapienza University of Rome. Baseline clinical and laboratory data, including TGA parameters, were collected, and patients were monitored for thrombotic events until death or last follow-up. TGA values at diagnosis displayed wide inter-individual and inter-subtype variability. With a median follow-up of 8.28 months, 8 (7.2%) thrombotic events were reported. No statistically significant association was found between baseline TGA parameters and the development of thrombotic events (p > 0.05). These findings suggest that a single TGA measurement at diagnosis may not predict thrombotic risk in AL patients. Future studies incorporating longitudinal TGA assessments and additional hemostatic evaluations, such as platelet function analysis, may help refine risk prediction for both thrombotic and hemorrhagic complications in this high-risk population.

Background: Acquired immunodeficiency syndrome (AIDS) caused by human immunodeficiency virus (HIV) remains a serious public health problem. Opportunistic infections and malignancies are the more frequent causes of hospitalization. We investigated hospitalized people with HIV (PWH) over the past 12 years to determine the types and changing trends of the disease presentation in a large tertiary academic centre in Eastern China.

Methods: We evaluated a total of 2,140 hospitalized PWH from January 2010 to December 2021. Demographic, clinical, and laboratory data, as well as opportunistic infections, malignancies, and in-hospital outcomes, were collected and analyzed.

Results: Over time, the incidence of opportunistic infections has declined. Conversely, the incidence of malignancies has increased, with non-AIDS-defining cancers (NADCs) occurring more frequently than ADCs. Notably, in 2020-2021, the incidence of NADCs surpassed that of opportunistic infections, marking a novel shift in the disease spectrum. The overall in-hospital mortality rate was 8.1%, and in-hospital mortality gradually decreased over time. Opportunistic infections, malignancies, and CD4⁺ T cell count were independent predictors of in-hospital mortality.

Conclusion: Our study provided a comprehensive description of the disease characteristics of PWH in eastern China over the past 12 years. The disease spectrum of PWH has undergone tremendous changes over time, highlighting the necessity of early HIV diagnosis and broader access to optimal treatment and management strategies.