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Outline of Iron Metabolism, with Emphasis on Erythroid Cells. 铁代谢概述,重点是红系细胞。
IF 1.5 4区 医学 Q3 HEMATOLOGY Pub Date : 2025-09-01 eCollection Date: 2025-01-01 DOI: 10.4084/MJHID.2025.067
Ugo Testa, Elvira Pelosi, Germana Castelli

Iron is required for several vital biological processes in all human cells. In mammals, a considerable number of proteins are involved in iron metabolism and utilize iron in many essential cellular processes, such as oxygen transport, mitochondrial respiration, gene regulation, and DNA synthesis or repair. Iron metabolism is a complex system finely regulated at both systemic and cellular levels. It involves the development of specialized mechanisms for iron absorption, transport, recycling, storage, and export, and protection against toxic compounds that can be generated during iron redox cycling in the presence of oxygen. The erythropoietic compartment consumes the majority of iron to support the high demand for hemoglobin synthesis. A tightly regulated system enables efficient iron uptake by erythroid cells and its subsequent processing for the synthesis of large amounts of heme, which is then incorporated into hemoglobin. A bidirectional regulatory system between erythropoiesis and iron metabolism ensures precise coordination between the two processes. This regulation is often disrupted in various anemic conditions.

铁是所有人体细胞中几个重要的生物过程所必需的。在哺乳动物中,相当数量的蛋白质参与铁代谢,并在许多基本的细胞过程中利用铁,如氧运输、线粒体呼吸、基因调控和DNA合成或修复。铁代谢是一个复杂的系统,在系统和细胞水平上都受到精细调节。它涉及铁的吸收、运输、回收、储存和出口的专门机制的发展,以及对在氧气存在的铁氧化还原循环过程中可能产生的有毒化合物的保护。红细胞生成室消耗大部分铁来支持血红蛋白合成的高需求。一个严格调控的系统使红细胞能够有效地摄取铁,并在随后的加工过程中合成大量的血红素,血红素随后被纳入血红蛋白。红细胞生成和铁代谢之间的双向调节系统确保了这两个过程之间的精确协调。这种调节在各种贫血情况下经常被破坏。
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引用次数: 0
From Legacy to Innovation: Pidotimod's Expanding Therapeutic Horizon. 从传统到创新:匹多莫德扩大治疗范围。
IF 1.5 4区 医学 Q3 HEMATOLOGY Pub Date : 2025-09-01 eCollection Date: 2025-01-01 DOI: 10.4084/MJHID.2025.057
Claudio Ucciferri, Francesca Chiappini, Jacopo Vecchiet, Katia Falasca

Pidotimod, a synthetic dipeptide, has been utilized for over three decades as an immunomodulatory agent to prevent recurrent respiratory infections, particularly in immunocompromised populations such as children and the elderly. Originally developed for its ability to enhance innate and adaptive immune responses, pidotimod is now being revisited in light of new clinical insights and emerging therapeutic needs. Recent studies have expanded its potential beyond traditional indications, with evidence supporting its role in patients with chronic respiratory diseases, such as chronic obstructive pulmonary disease (COPD), allergic rhinitis, and even viral infections, including SARS-CoV-2. Pidotimod exerts its effects by stimulating dendritic cells, enhancing toll-like receptor (TLR) expression, and promoting cytokine production, including IL-2 and IFN-γ, thereby supporting both cellular and humoral immunity. This broad-spectrum immune modulation makes pidotimod a promising adjunct in managing immune-mediated diseases and infections in both immunocompetent and immunocompromised individuals. In this review, we examine pidotimod's pharmacodynamics, summarize clinical evidence from recent studies, and explore its evolving role in modern therapeutic strategies for infectious diseases. Given its safety profile and oral administration, pidotimod holds significant promise not only for preventing infections but also as part of a broader immunomodulatory approach in complex disease management.

匹多莫德是一种合成的二肽,作为免疫调节剂用于预防复发性呼吸道感染已有30多年的历史,特别是在免疫功能低下的人群中,如儿童和老年人。匹多莫德最初是为了增强先天和适应性免疫反应而开发的,现在正在根据新的临床见解和新出现的治疗需求重新审视。最近的研究已将其潜力扩展到传统适应症之外,有证据支持其在慢性呼吸道疾病患者中的作用,如慢性阻塞性肺疾病(COPD)、过敏性鼻炎,甚至包括SARS-CoV-2在内的病毒感染。匹多莫德通过刺激树突状细胞、增强toll样受体(TLR)表达、促进细胞因子(包括IL-2和IFN-γ)的产生来发挥作用,从而支持细胞和体液免疫。这种广谱免疫调节使匹多莫德在免疫功能正常和免疫功能低下的个体中成为一种有希望的治疗免疫介导的疾病和感染的辅助药物。在这篇综述中,我们研究了匹多莫德的药效学,总结了最近研究的临床证据,并探讨了它在现代传染病治疗策略中的作用。鉴于其安全性和口服给药,匹多莫德不仅在预防感染方面具有重要的前景,而且在复杂疾病管理中作为更广泛的免疫调节方法的一部分。
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引用次数: 0
Prevention of Early Death in Very Elderly Acute Promyelocytic Leukemia Patients Using Lower Induction Doses of All-trans Retinoic Acid. 低诱导剂量全反式维甲酸预防高龄急性早幼粒细胞白血病患者早期死亡
IF 1.5 4区 医学 Q3 HEMATOLOGY Pub Date : 2025-09-01 eCollection Date: 2025-01-01 DOI: 10.4084/MJHID.2025.066
Mark Dalgetty, Sruthi Dontu, Chandini Kannan, Amany Keruakous, Locke Bryan, Vamsi Kota, Anand Jillella
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引用次数: 0
Assessment of the Efficacy of Procalcitonin, C-Reactive Protein, and Albumin Levels-guided Antibiotics Use in Sepsis. 评估降钙素原、c反应蛋白和白蛋白水平引导抗生素在败血症中的应用效果。
IF 1.5 4区 医学 Q3 HEMATOLOGY Pub Date : 2025-09-01 eCollection Date: 2025-01-01 DOI: 10.4084/MJHID.2025.060
Junyue Cao, Yuchao Sun

Background: Efficient management of sepsis requires precise antibiotic therapy. This study examines the efficacy of guiding such therapy using Procalcitonin (PCT), C-Reactive Protein (CRP), and albumin levels.

Methods: A total of 127 adult sepsis patients were assigned to either standard care or a biomarker-guided group where antibiotic use was adjusted based on biomarker levels.

Results: The biomarker-guided approach significantly reduced the duration of antibiotic therapy (9.0 vs. 10.5 days, P=0.04) and expedited antibiotic de-escalation. Hospital costs were lower in the biomarker-guided group (20,000 vs. 24,000 CNY, P=0.04), though reductions in secondary infections did not reach statistical significance. There was no significant difference in 28-day mortality rates.

Conclusion: Biomarker-guided antibiotic therapy can enhance the efficiency of treatment in sepsis, reducing both duration and cost without impacting patient safety. These findings suggest the potential benefits of incorporating biomarkers into standard sepsis management protocols.

背景:有效的脓毒症管理需要精确的抗生素治疗。本研究考察了使用降钙素原(PCT)、c反应蛋白(CRP)和白蛋白水平指导此类治疗的效果。方法:共有127名成年脓毒症患者被分配到标准护理组或生物标志物指导组,根据生物标志物水平调整抗生素使用。结果:生物标志物引导的方法显着缩短了抗生素治疗的持续时间(9.0天对10.5天,P=0.04)并加速了抗生素的降级。生物标志物引导组的住院费用较低(2万元对2.4万元,P=0.04),但继发感染的减少没有达到统计学意义。28天死亡率无显著差异。结论:生物标志物引导的抗生素治疗可提高脓毒症的治疗效率,在不影响患者安全的情况下减少持续时间和成本。这些发现表明将生物标志物纳入标准脓毒症管理方案的潜在益处。
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引用次数: 0
Late-Onset Veno-Occlusive Disease (VOD) Post Haploidentical Transplant: A Diagnostic Challenge Requiring Liver Biopsy- A Case Report. 单倍体移植后迟发性静脉闭塞疾病(VOD):需要肝活检的诊断挑战-一个病例报告。
IF 1.5 4区 医学 Q3 HEMATOLOGY Pub Date : 2025-09-01 eCollection Date: 2025-01-01 DOI: 10.4084/MJHID.2025.068
Mehak Trehan, Homdutt Sharma, Abhinav Sengupta, Anirudh Burli, Pradeep Kumar, Manoranjan Mahapatra
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引用次数: 0
Health-Related Quality of Life Measurement in Adults With Sickle Cell Disease in Steady State: Experience of One French Reference Center. 稳定状态成人镰状细胞病患者的健康相关生活质量测量:一个法国参考中心的经验
IF 1.5 4区 医学 Q3 HEMATOLOGY Pub Date : 2025-09-01 eCollection Date: 2025-01-01 DOI: 10.4084/MJHID.2025.065
Giovanna Cannas, Solène Poutrel, Emilie Virot, Manon Marie, Alexandre Guilhem, Amal El-Kanouni, Richard Bourgeay, Marie-Grace Mutumwa, Mohamed Elhamri, Arnaud Hot

Sickle cell disease (SCD) is a genetic disease of public health concern. Adult patients face various disease-related complications, which affect their quality of life (QoL). Few studies have examined relationships between these events and health-related (HR) QoL. We conducted a study of 240 adults with SCD seen in steady state at a routine clinic visit over one year. Two self-administered questionnaires were used to determine patients' HRQoL: the sickle cell self-efficacy scale (SCSES) comprising 9 specific items and the unspecific SF-36 scoring system comprising 8 subscales, which construct the physical component summary (PCS) and the mental component summary (MCS). Factors impacting HRQoL were established using univariate and multivariate regression analyses. Participants had a median age of 28 years (Sex ratio male/female 0.61; 68% SS genotype). Most of them had experienced more than one SCD-related complication and more than one affected organ system. A good correlation was established between the SCD-specific and the unspecific scoring systems (p < 0.0001). Using the SF-36 scoring system, energy/fatigue, general health, and pain subscales showed the lowest median scores (50, 45, and 56.5, respectively), while physical functioning had the highest median score (75). In univariate and multivariate analyses, hospitalization for SCD complications occurring during the last year preceding QoL evaluation was the main feature impacting HRQoL (p < 0.001). Good compliance to hydroxyurea (HU) therapy was associated with higher SCSES (p = 0.04) and higher emotional role functioning (p = 0.03) scores. The recent occurrence of severe SCD complications mainly influenced HRQoL. Our study suggests that a more effective treatment through better compliance with HU therapy would provide benefit in terms of QoL.

镰状细胞病(SCD)是一种引起公共卫生关注的遗传性疾病。成年患者面临各种疾病相关并发症,影响其生活质量。很少有研究调查这些事件与健康相关生活质量之间的关系。我们对240名成年SCD患者进行了一项研究,这些患者在一年多的时间里在常规诊所就诊时处于稳定状态。采用镰状细胞自我效能量表(SCSES)和非特异性SF-36评分系统(8个子量表,分别构成生理成分摘要(PCS)和心理成分摘要(MCS))两种自填问卷来确定患者的HRQoL。采用单因素和多因素回归分析确定影响HRQoL的因素。参与者的中位年龄为28岁(性别比男女0.61;68% SS基因型)。大多数患者都经历过不止一种scd相关并发症和不止一种受影响的器官系统。scd特异性评分系统与非特异性评分系统之间存在良好的相关性(p < 0.0001)。使用SF-36评分系统,能量/疲劳、一般健康和疼痛亚量表的中位数得分最低(分别为50、45和56.5),而身体功能的中位数得分最高(75)。在单因素和多因素分析中,生活质量评估前一年发生的SCD并发症住院是影响HRQoL的主要特征(p < 0.001)。羟基脲(HU)治疗的良好依从性与较高的SCSES (p = 0.04)和较高的情绪角色功能(p = 0.03)评分相关。近期发生的严重SCD并发症主要影响HRQoL。我们的研究表明,通过更好地依从HU治疗,更有效的治疗将在生活质量方面提供益处。
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引用次数: 0
Atypical Dermatological Manifestations of Syphilis in Advanced HIV: A Case-Based Narrative Review. 晚期HIV患者梅毒的非典型皮肤病学表现:一项基于病例的叙述综述。
IF 1.5 4区 医学 Q3 HEMATOLOGY Pub Date : 2025-09-01 eCollection Date: 2025-01-01 DOI: 10.4084/MJHID.2025.056
Irene Scarvaglieri, Federico Cesanelli, Giorgio Tiecco, Iacopo Ghini, Davide Minisci, Caterina Davoli, Stefano Rapino, Emanuele Focà, Maria Alberti, Martina Salvi, Francesco Castelli, Eugenia Quiros-Roldan

Syphilis, a sexually transmitted infection caused by Treponema pallidum (T. pallidum), is re-emerging globally. Recent epidemiological data show a rising incidence, particularly among men who have sex with men (MSM). Known as 'the great mimic' for its broad clinical spectrum, secondary syphilis classically presents with a maculopapular rash involving the trunk and extremities. However, it can also present with atypical cutaneous manifestations, especially in immunocompromised patients. This aspect may contribute to delayed diagnosis and treatment. Starting from a clinical case, we will conduct a literature review on syphilis/HIV coinfection, with a particular focus on the broad spectrum of cutaneous manifestations and the key differential diagnoses involved. We report the case of a 60-year-old male living with HIV who presented with non-pruritic, polymorphic skin lesions sparing the palms and soles. The patient had a prior history of treated latent syphilis. Initial diagnostic workup excluded common differentials, including monkeypox and fungal infections. Serologic testing confirmed active syphilis with a reactive Rapid Plasma Reagin (RPR) titer of 1:32, skin biopsy showed dense plasma cell-rich infiltrate, and immunohistochemistry was positive for T. pallidum. Despite negative cerebrospinal fluid findings, neurological symptoms prompted treatment with intravenous penicillin G, and the symptoms resolved with treatment. This case underscores the importance of considering syphilis in the differential diagnosis of atypical dermatologic presentations, given its increasing prevalence and potential for severe systemic involvement.

梅毒,一种由梅毒螺旋体(T. pallidum)引起的性传播感染,正在全球重新出现。最近的流行病学数据显示,发病率正在上升,特别是在男男性行为者(MSM)中。因其广泛的临床范围而被称为“伟大的模仿者”,继发性梅毒的典型表现是累及躯干和四肢的斑疹。然而,它也可以呈现非典型的皮肤表现,特别是在免疫功能低下的患者。这方面可能导致延误诊断和治疗。我们将从一个临床病例开始,对梅毒/HIV合并感染的文献进行综述,特别关注广泛的皮肤表现和涉及的关键鉴别诊断。我们报告的情况下,60岁的男性艾滋病毒感染者谁提出了非瘙痒,多态皮肤病变保留手掌和鞋底。患者既往有潜伏性梅毒治疗史。初步诊断排除了常见的差异,包括猴痘和真菌感染。血清学检查证实活动性梅毒,反应性快速血浆反应素(RPR)滴度为1:32,皮肤活检显示密集的富含浆细胞浸润,免疫组织化学检测梅毒T.阳性。尽管脑脊液检查呈阴性,但神经系统症状提示静脉注射青霉素G治疗,治疗后症状消失。该病例强调了在非典型皮肤病表现的鉴别诊断中考虑梅毒的重要性,因为梅毒的患病率越来越高,并有可能严重累及全身。
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引用次数: 0
Zanubrutinib as Upfront Treatment in de Novo B-Cell Prolymphocytic Leukemia: The Case of Two Elderly Patients. 扎鲁替尼作为新发b细胞前淋巴细胞白血病的前期治疗:两例老年患者的病例分析。
IF 1.5 4区 医学 Q3 HEMATOLOGY Pub Date : 2025-09-01 eCollection Date: 2025-01-01 DOI: 10.4084/MJHID.2025.061
Silvia Giorgi, Giovanna Leonardi, Monica Maccaferri, Elena Sbadili, Carlo Caterina, Giovanni Fera, Ambra Paolini, Leonardo Potenza, Anna Candoni, Mario Luppi, Roberto Marasca
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引用次数: 0
Predisposing Factors to Infections in Thalassemia Syndrome Patients. 地中海贫血综合征患者感染的易感因素。
IF 1.5 4区 医学 Q3 HEMATOLOGY Pub Date : 2025-09-01 eCollection Date: 2025-01-01 DOI: 10.4084/MJHID.2025.055
Eman M Mansory, Lina M Abdulrahman, Balsam Osman, Sawsan M Alsiyoufi, Assil F Ruckn, Marwa Aljedaani, Nemmat Hassan, Ahmed S Barefah, Hatem M Alahwal, Yassir Daghistani, Salem M Bahashwan, Abdullah T Almohammadi, Osman O Radhwi

Background: Thalassemia is an inherited blood disorder characterized by abnormal hemoglobin production, leading to chronic anemia. Patients, particularly those who are transfusion-dependent, face a heightened risk of infections due to disease-related factors like anemia and treatment-related complications such as iron overload and splenectomy. This study explores the factors contributing to infections in thalassemia patients to improve management strategies.

Methods: A retrospective analysis was conducted on 303 patients with thalassemia at a tertiary care center from 2007 to 2022. Data were collected on demographics, transfusion dependency, splenectomy status, ferritin levels, vaccination history, and culture results. Logistic regression analysis was used to identify infection risk factors, with significance set at p-value < 0.05.

Results: Out of 303 patients, 72 (23.8%) experienced culture-positive infections, with Escherichia coli being the most isolated pathogen. Patients with infections had significantly higher ferritin levels and were less likely to be on chelation therapy. Chelation therapy was significantly associated with a reduced risk of infection (OR 0.18, p < 0.001). Higher serum albumin levels were also associated with lower odds of infection (OR 0.92, p < 0.001). Mortality was significantly higher among patients with positive cultures compared to those without (22% vs. 3%, p < 0.001).

Conclusion: This study highlights the strong link between iron overload, chelation, and risk of infection in thalassemia patients. Effective management, including proper chelation therapy and monitoring ferritin levels, is critical for reducing infections and improving outcomes. Further studies are needed to confirm these findings and guide future management strategies.

背景:地中海贫血是一种以血红蛋白生成异常为特征的遗传性血液疾病,可导致慢性贫血。患者,特别是那些依赖输血的患者,由于疾病相关因素(如贫血)和治疗相关并发症(如铁超载和脾切除术)而面临更高的感染风险。本研究旨在探讨导致地中海贫血患者感染的因素,以改善管理策略。方法:对2007 ~ 2022年在某三级保健中心就诊的303例地中海贫血患者进行回顾性分析。收集的数据包括人口统计学、输血依赖、脾切除术状态、铁蛋白水平、疫苗接种史和培养结果。采用Logistic回归分析确定感染危险因素,p值< 0.05。结果:303例患者中,培养阳性72例(23.8%),其中大肠杆菌是分离最多的病原体。感染患者的铁蛋白水平明显较高,接受螯合治疗的可能性较小。螯合治疗与感染风险降低显著相关(OR 0.18, p < 0.001)。较高的血清白蛋白水平也与较低的感染几率相关(OR 0.92, p < 0.001)。培养阳性患者的死亡率明显高于未培养阳性患者(22% vs. 3%, p < 0.001)。结论:本研究强调了地中海贫血患者铁超载、螯合和感染风险之间的密切联系。有效的管理,包括适当的螯合治疗和监测铁蛋白水平,对于减少感染和改善结果至关重要。需要进一步的研究来证实这些发现并指导未来的管理策略。
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引用次数: 0
Luspatercept Treatment in a β-Thalassemia Patient with Pulmonary Arterial Hypertension: A Case Report. Luspatercept治疗β-地中海贫血合并肺动脉高压1例。
IF 1.5 4区 医学 Q3 HEMATOLOGY Pub Date : 2025-09-01 eCollection Date: 2025-01-01 DOI: 10.4084/MJHID.2025.059
Beibei Yang, Dongmei Liu, Changyu Yang, Yali Zhou, Guiping Liao, Jian Huang, Yingying Li, Yinjiang Tang, Xiaolin Yin
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引用次数: 0
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Mediterranean Journal of Hematology and Infectious Diseases
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