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Goal-Directed Use of Prothrombin Complex Concentrates in Liver Transplantation: Is a Plasma-Free Procedure Feasible? 肝移植中凝血酶原复合物浓缩物的目标导向使用:无血浆手术可行吗?
IF 1.1 Q4 HEMATOLOGY Pub Date : 2024-07-08 DOI: 10.3390/hematolrep16030044
G. Punzo, Valeria Di Franco, P. Aceto
Background: Fresh frozen plasma (FFP) transfusions have been the mainstay of hemostatic intervention for the treatment of bleeding and coagulation abnormalities arising during liver transplantation (LT) for decades. However, numerous clinical studies showed that FFP has many side effects, including the risk of pathogen transmission, transfusion-associated circulatory overload (TACO), transfusion-related immunomodulation (TRIM), and transfusion-related acute lung injury (TRALI). These adverse events are particularly challenging in patients undergoing LT, who often suffer from severe portal hypertension, poor renal function and coexisting cardiac disease.The aims of this review are to summarize the pharmacological properties of currently available PCCs, to represent the theoretical benefits and the possible risks related to the use of these drugs in patients undergoing LT, and, finally, to review the current literature on the topic in order to highlight the evidence that currently supports PCC use in LT patients. Methods: The current literature on the topic was reviewed in order to highlight the evidence that currently supports PCC use in LT patients. Results: Prothrombin complex concentrates (PCCs) may offer several advantages when compared to FFP. Indeed, PCCs have been shown to reduce the risk of TACO, which during liver transplantation may deteriorate portal hypertension, increase intraoperative bleeding, and possibly reduce survival rates. One of the major concerns for PCC use is thrombogenicity. However, currently available PCCs are much safer as they contain inactivated forms of the vitamin K-dependent coagulation factors and protein C, protein S, antithrombin and/or heparin. Nowadays, the use of PCCs to correct coagulation abnormalities that occur during LT is an increasingly widespread practice. However, it is not yet clear what level of evidence supports this practice, and what the risks associated with it are. Conclusions: Administration of PCC in LT patients to correct haemostatic abnormalities seems to be well-tolerated, but the relationship between PCC use and thromboembolic events in the postoperative period remains unclear. Adequately powered, methodologically sound trials are urgently required for more definitive conclusions about the efficacy and safety of PCCs in a broad phenotype of LT recipients.
背景:几十年来,新鲜冰冻血浆(FFP)输注一直是治疗肝移植(LT)过程中出血和凝血异常的主要止血手段。然而,大量临床研究表明,FFP 有许多副作用,包括病原体传播风险、输血相关循环超负荷(TACO)、输血相关免疫调节(TRIM)和输血相关急性肺损伤(TRALI)。本综述旨在总结目前可用的 PCC 的药理特性,说明在接受 LT 的患者中使用这些药物的理论益处和可能存在的风险,最后回顾目前有关该主题的文献,以强调目前支持在 LT 患者中使用 PCC 的证据。方法:回顾当前有关该主题的文献,以强调目前支持在 LT 患者中使用 PCC 的证据。结果:凝血酶原复合物浓缩物 (PCC) 与全血细胞生成素 (FFP) 相比具有多项优势。事实上,PCC 已被证明可降低 TACO 的风险,而在肝移植过程中,TACO 可能会恶化门静脉高压,增加术中出血,并可能降低存活率。使用 PCC 的主要顾虑之一是血栓形成性。不过,目前可用的 PCC 更加安全,因为它们含有灭活形式的维生素 K 依赖性凝血因子、蛋白 C、蛋白 S、抗凝血酶和/或肝素。如今,使用 PCC 来纠正 LT 期间出现的凝血异常已成为一种越来越普遍的做法。然而,目前尚不清楚支持这种做法的证据水平以及与之相关的风险。结论:在LT患者中使用PCC来纠正止血异常似乎具有良好的耐受性,但PCC的使用与术后血栓栓塞事件之间的关系仍不清楚。要想就 PCC 对多种表型的 LT 受者的疗效和安全性得出更明确的结论,亟需进行动力充足、方法可靠的试验。
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引用次数: 0
Blood Microbiota and Its Products: Mechanisms of Interference with Host Cells and Clinical Outcomes 血液微生物群及其产物:干扰宿主细胞的机制与临床结果
IF 1.1 Q4 HEMATOLOGY Pub Date : 2024-07-06 DOI: 10.3390/hematolrep16030043
L. Santacroce, I. Charitos, Marica Colella, Raffele Palmirotta, Emilio Jirillo
In healthy conditions, blood was considered a sterile environment until the development of new analytical approaches that allowed for the detection of circulating bacterial ribosomal DNA. Currently, debate exists on the origin of the blood microbiota. According to advanced research using dark field microscopy, fluorescent in situ hybridization, flow cytometry, and electron microscopy, so-called microbiota have been detected in the blood. Conversely, others have reported no evidence of a common blood microbiota. Then, it was hypothesized that blood microbiota may derive from distant sites, e.g., the gut or external contamination of blood samples. Alteration of the blood microbiota’s equilibrium may lead to dysbiosis and, in certain cases, disease. Cardiovascular, respiratory, hepatic, kidney, neoplastic, and immune diseases have been associated with the presence of Gram-positive and Gram-negative bacteria and/or their products in the blood. For instance, lipopolysaccharides (LPSs) and endotoxins may contribute to tissue damage, fueling chronic inflammation. Blood bacteria can interact with immune cells, especially with monocytes that engulf microorganisms and T lymphocytes via spontaneous binding to their membranes. Moreover, LPSs, extracellular vesicles, and outer membrane vesicles interact with red blood cells and immune cells, reaching distant organs. This review aims to describe the composition of blood microbiota in healthy individuals and those with disease conditions. Furthermore, special emphasis is placed on the interaction of blood microbiota with host cells to better understand disease mechanisms.
在健康状况下,血液被认为是无菌环境,直到新分析方法的出现,可以检测循环细菌核糖体 DNA。目前,关于血液微生物群的起源还存在争议。根据利用暗视野显微镜、荧光原位杂交、流式细胞术和电子显微镜进行的先进研究,在血液中检测到了所谓的微生物群。与此相反,另一些研究报告则称没有证据表明存在常见的血液微生物群。因此,有人假设血液微生物群可能来自远处,如肠道或血液样本的外部污染。血液微生物群平衡的改变可能导致菌群失调,并在某些情况下引发疾病。心血管、呼吸、肝脏、肾脏、肿瘤和免疫疾病都与血液中存在革兰氏阳性和革兰氏阴性细菌和/或其产物有关。例如,脂多糖(LPSs)和内毒素可能会造成组织损伤,加剧慢性炎症。血液中的细菌可与免疫细胞相互作用,特别是与吞噬微生物的单核细胞和自发结合到其膜上的 T 淋巴细胞。此外,LPSs、细胞外囊泡和外膜囊泡还会与红细胞和免疫细胞相互作用,到达远处的器官。本综述旨在描述健康人和疾病患者血液微生物群的组成。此外,文章还特别强调了血液微生物群与宿主细胞的相互作用,以便更好地了解疾病机制。
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引用次数: 0
Transient Stress Lymphocytosis in a Child: A Case Report and Systematic Review of the Literature 儿童短暂性应激淋巴细胞增多症:病例报告和文献系统回顾
IF 1.1 Q4 HEMATOLOGY Pub Date : 2024-07-03 DOI: 10.3390/hematolrep16030042
Alexander Placek, Randall Y. Chan, Maria Vergara-Lluri, Russell K. Brynes
Transient stress lymphocytosis (TSL) is an under-recognized phenomenon associated with an acute stressful event such as physical trauma or various emergency medical conditions. Lymphocytosis generally resolves within several hours to days of the stressor. While most reports of TSL predominantly involve adult patients, it has only rarely been reported in pediatric patients. Here, we describe the clinical course of a 9-year-old male who developed TSL following a traumatic fall from a second-story balcony and provide a systematic literature review of TSL.
一过性应激淋巴细胞增多症(TSL)是与急性应激事件(如身体创伤或各种紧急医疗状况)相关的一种未得到充分认识的现象。淋巴细胞增多通常在应激事件发生后数小时至数天内消退。大多数关于 TSL 的报道主要涉及成年患者,而关于儿童患者的报道却很少。在这里,我们描述了一名 9 岁男性患者从二楼阳台跌落后出现 TSL 的临床过程,并对 TSL 进行了系统的文献综述。
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引用次数: 0
Double Trouble: COVID-19 Infection Exacerbates Sickle Cell Crisis Outcomes in Hospitalized Patients-Insights from National Inpatient Sample 2020. 双重麻烦:COVID-19感染加剧住院患者镰状细胞危象--来自2020年全国住院患者样本的观察。
IF 1.1 Q4 HEMATOLOGY Pub Date : 2024-06-29 DOI: 10.3390/hematolrep16030041
Zubair Hassan Bodla, Mariam Hashmi, Fatima Niaz, Austin B Auyeung, Anuoluwa Oyetoran, Muhammad Jahanzeb Khalil, Muhammad Salman Faisal, Farhan Khalid, Abdel-Rahman Zakieh, Yvette Bazikian, Christopher L Bray

Background: This study investigated the impact of COVID-19 on patients with sickle cell crisis (SCC) using National Inpatient Sample (NIS) data for the year 2020. Methods: A retrospective cohort analysis was conducted utilizing International Classification of Diseases (ICD-10) codes to identify adults who were admitted with a principal diagnosis of sickle cell crisis. The primary outcomes examined were inpatient mortality, while the secondary outcomes assessed included morbidity, hospital length of stay, and resource utilization. Analyses were conducted with STATA. Multivariate logistic and linear regression analyses were used to adjust for confounding variables. Results: Of 66,415 adult patients with a primary SCC diagnosis, 875 were identified with a secondary diagnosis of COVID-19 infection. Unadjusted mortality rate was higher for SCC patients with COVID-19 (2.28%) compared to those without (0.33%), with an adjusted odds ratio (aOR) of 8.49 (p = 0.001). They also showed increased odds of developing acute respiratory failure (aOR = 2.37, p = 0.003) and acute kidney injury requiring dialysis (aOR = 8.66, p = 0.034). Additionally, these patients had longer hospital stays by an adjusted mean of 3.30 days (p < 0.001) and incurred higher hospitalization charges by an adjusted mean of USD 35,578 (p = 0.005). Conclusions: The SCC patients with COVID-19 presented higher mortality rates, increased morbidity indicators, longer hospital stays, and substantial economic burdens.

背景:本研究利用 2020 年全国住院病人样本 (NIS) 数据,调查 COVID-19 对镰状细胞危象(SCC)患者的影响。研究方法利用国际疾病分类(ICD-10)代码进行回顾性队列分析,以确定以镰状细胞危象为主要诊断入院的成年人。研究的主要结果是住院病人死亡率,次要结果包括发病率、住院时间和资源利用率。分析采用 STATA 软件进行。多变量逻辑分析和线性回归分析用于调整混杂变量。结果:在 66,415 名确诊为原发性 SCC 的成人患者中,有 875 人被确诊为 COVID-19 感染的继发性患者。感染 COVID-19 的 SCC 患者未经调整的死亡率(2.28%)高于未感染者(0.33%),调整后的几率比(aOR)为 8.49(p = 0.001)。他们发生急性呼吸衰竭(aOR = 2.37,p = 0.003)和需要透析的急性肾损伤(aOR = 8.66,p = 0.034)的几率也有所增加。此外,这些患者的住院时间更长,调整后的平均住院时间为 3.30 天(p < 0.001),住院费用更高,调整后的平均住院费用为 35,578 美元(p = 0.005)。结论患有 COVID-19 的 SCC 患者死亡率更高、发病率指标更高、住院时间更长、经济负担更大。
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引用次数: 0
A Rare Onset of T-Lymphoid Blast Crisis in Chronic Myeloid Leukemia with Two Distinct Blast Populations. 慢性髓性白血病中罕见的 T 淋巴细胞暴发危象与两种不同的暴发群
IF 1.1 Q4 HEMATOLOGY Pub Date : 2024-06-27 DOI: 10.3390/hematolrep16030040
Alessandra Mongia, Francesca Romano, Sara Ciullini Mannurita, Benedetta Peruzzi, Sara Bencini, Daniela Parrini, Laura Fasano, Alessandra Fanelli

Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm characterized by bone marrow expansion and the proliferation of one or more myeloid cell lineages, predominantly driven by the expression of the constitutively active fusion product tyrosine kinase BCR:ABL1. Rarely, CML patients directly develop a blast crisis (BC), mostly of myeloid origin. CML at blast crisis with a T-cell phenotype at diagnosis, without any prior history of CML, is extremely rare. Herein, we describe one rare CML case, in a young man showing an unusual and early T-lymphoid blastic crisis at diagnosis, as the first onset of a previously unknown CML. The multidisciplinary collaboration between laboratorians and clinicians for the diagnosis and management of this atypical case was crucial in outlining both a targeted pharmacological treatment and a successful hematopoietic stem cell transplantation.

慢性髓性白血病(CML)是一种骨髓增生性肿瘤,其特点是骨髓增生和一个或多个髓系细胞增殖,主要由组成型活性融合产物酪氨酸激酶 BCR:ABL1 的表达驱动。极少数情况下,CML 患者会直接出现鼓风危象 (BC),多数为髓系细胞来源。诊断时具有 T 细胞表型的爆发危象 CML,且之前没有任何 CML 病史,这种情况极为罕见。在此,我们描述了一个罕见的 CML 病例,患者是一名年轻男性,诊断时表现出不寻常的早期 T 淋巴细胞暴发性危象,是之前未知的 CML 首次发病。在诊断和处理这一非典型病例的过程中,实验室医生和临床医生之间的多学科合作对于制定有针对性的药物治疗方案和成功进行造血干细胞移植至关重要。
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引用次数: 0
Treatment of Immune Thrombocytopenia: Contextualization from a Historical Perspective. 免疫性血小板减少症的治疗:从历史角度看背景。
IF 1.1 Q4 HEMATOLOGY Pub Date : 2024-06-26 DOI: 10.3390/hematolrep16030039
Daniel Martínez-Carballeira, Ángel Bernardo, Alberto Caro, Inmaculada Soto, Laura Gutiérrez

Immune thrombocytopenia (ITP) is an autoimmune disease characterized by an isolated decrease in platelet count and an increased risk of bleeding. The pathogenesis is complex, affecting multiple components of the immune system and causing both peripheral destruction of platelets and inadequate production in the bone marrow. In this article, we review the treatment of ITP from a historical perspective, discussing first line and second line treatments, and management of refractory disease.

免疫性血小板减少症(ITP)是一种自身免疫性疾病,其特点是血小板数量减少和出血风险增加。其发病机制复杂,影响免疫系统的多个组成部分,导致血小板外周破坏和骨髓生成不足。本文从历史角度回顾了 ITP 的治疗,讨论了一线和二线治疗以及难治性疾病的处理。
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引用次数: 0
Neutropenia in Childhood-A Narrative Review and Practical Diagnostic Approach. 儿童中性粒细胞减少症--叙事回顾与实用诊断方法。
IF 1.1 Q4 HEMATOLOGY Pub Date : 2024-06-16 DOI: 10.3390/hematolrep16020038
Georgios Katsaras, Silouani Koutsi, Evdokia Psaroulaki, Dimitra Gouni, Pelagia Tsitsani

Neutropenia refers to a decrease in the absolute neutrophil count according to age and race norms and poses a common concern in pediatric practice. Neutrophils serve as host defenders and act crucially in acute inflammation procedures. In this narrative review, we systematically present causes of neutropenia in childhood, mainly adopting the pathophysiological classification of Frater, thereby studying (1) neutropenia with reduced bone marrow reserve, (2) secondary neutropenia with reduced bone marrow reserve, and (3) neutropenia with normal bone marrow reserve. Different conditions in each category are thoroughly discussed and practically approached from the clinician's point of view. Secondary mild to moderate neutropenia is usually benign due to childhood viral infections and is expected to resolve in 2-4 weeks. Bacterial and fungal agents are also associated with transient neutropenia, although fever with severe neutropenia constitutes a medical emergency. Drug-induced and immune neutropenias should be suspected following a careful history and a detailed clinical examination. Cytotoxic chemotherapies treating malignancies are responsible for severe neutropenia and neutropenic shock. Rare genetic neutropenias usually manifest with major infections early in life. Our review of taxonomies clinical findings and associates them to specific neutropenia disorders. We consequently propose a practical diagnostic algorithm for managing neutropenic children.

中性粒细胞减少症是指根据年龄和种族标准,中性粒细胞绝对计数减少,是儿科常见的问题。中性粒细胞是宿主的保卫者,在急性炎症过程中起着至关重要的作用。在这篇叙述性综述中,我们系统地介绍了儿童期中性粒细胞减少症的病因,主要采用了弗雷特的病理生理学分类法,从而研究了(1)骨髓储备减少的中性粒细胞减少症;(2)骨髓储备减少的继发性中性粒细胞减少症;以及(3)骨髓储备正常的中性粒细胞减少症。从临床医生的角度出发,对每个类别中的不同情况进行了深入讨论和实际操作。继发性轻度至中度中性粒细胞减少症通常是良性的,由儿童病毒感染引起,预计会在 2-4 周内缓解。细菌和真菌感染也会导致一过性中性粒细胞减少症,但发热伴严重中性粒细胞减少症属于急症。在仔细询问病史和进行详细的临床检查后,应怀疑是药物引起的中性粒细胞减少症和免疫性中性粒细胞减少症。治疗恶性肿瘤的细胞毒性化疗可导致严重的中性粒细胞减少症和中性粒细胞减少性休克。罕见的遗传性中性粒细胞减少症通常表现为生命早期的重大感染。我们的综述对临床发现进行了分类,并将其与特定的中性粒细胞减少症联系起来。因此,我们提出了一种实用的诊断算法,用于治疗中性粒细胞减少症患儿。
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引用次数: 0
Three-Way Translocation t(12;15;17) (p13;q24;q21) Found in Acute Promyelocytic Leukemia with Basophilic Differentiation 在嗜碱性分化的急性早幼粒细胞白血病中发现三向易位 t(12;15;17) (p13;q24;q21)
IF 0.9 Q4 Medicine Pub Date : 2024-06-12 DOI: 10.3390/hematolrep16020037
Sara Frazzetto, Lara Gullo, Gabriele Sapuppo, Manlio Fazio, Cristina Lo Faro, Giuliana Giunta, Ignazio Caravotta, Elisa Mauro, M. Parisi, A. Triolo, N. Parrinello, Maria Letizia Consoli, Loredana També, D. Cambria, Sara Marino, Grazia Scuderi, F. Di Raimondo
Acute promyelocytic leukemia is a rare form of acute myeloid leukemia in which immature promyelocytes abnormally proliferate in the bone marrow. In most cases, the disease is characterised by the translocation t(15;17) (q24;q21), which causes the formation of PML::RARA, an oncogenic fusion protein responsible for blocking myeloid differentiation and survival advantage. Here, we present a case of acute promyelocytic leukemia with two unusual features: basophilic differentiation and a three-way translocation involving chromosomes 12, 15 and 17. In the few cases reported, basophilic differentiation was associated with a poor prognosis. In contrast, our patient responded promptly to the standard treatment with all-trans-retinoic acid (ATRA) and arsenic trioxide (ATO) and obtained complete remission. To our knowledge, this is the first report of basophilic acute promyelocytic leukemia with the three-way translocation t(12;17;15) (p13; q24;q21).
急性早幼粒细胞白血病是一种罕见的急性髓系白血病,未成熟的早幼粒细胞在骨髓中异常增殖。在大多数病例中,该病的特征是t(15;17) (q24;q21)易位,它导致PML::RARA的形成,PML::RARA是一种致癌融合蛋白,负责阻碍髓系分化和生存优势。在此,我们介绍了一例急性早幼粒细胞白血病病例,该病例具有两个不同寻常的特征:嗜碱性分化和涉及 12、15 和 17 号染色体的三向易位。在少数报道的病例中,嗜碱性分化与预后不良有关。相比之下,我们的患者对全反式维甲酸(ATRA)和三氧化二砷(ATO)的标准治疗反应迅速,病情完全缓解。据我们所知,这是首次报道嗜碱性急性早幼粒细胞白血病伴有三向易位 t(12;17;15) (p13; q24;q21)。
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引用次数: 0
Skin Hypopigmentation in Hematology Disorders 血液病中的皮肤色素沉着
IF 0.9 Q4 Medicine Pub Date : 2024-06-04 DOI: 10.3390/hematolrep16020036
Roberto Mazzetto, Paola Miceli, A. Sernicola, Jacopo Tartaglia, Mauro Alaibac
Hypopigmentation disorders pose significant diagnostic challenges in dermatology, sometimes reflecting underlying hematological conditions. This review explores the clinical presentations related to hypopigmentation in hematological disorders, focusing on vitiligo, morphea, and syndromic albinism. Vitiligo, an autoimmune disorder targeting melanocytes, involves interactions between genetic polymorphisms and immune responses, particularly regarding CD8+ T cells and IFN-γ. Drug-induced vitiligo, notably by immune checkpoint inhibitors and small-molecule targeted anticancer therapies, underscores the importance of immune dysregulation. Morphea, an inflammatory skin disorder, may signal hematological involvement, as seen in deep morphea and post-radiotherapy lesions. Syndromic albinism, linked to various genetic mutations affecting melanin production, often presents with hematologic abnormalities. Treatment approaches focus on targeting the immune pathways specific to the condition, and when that is not possible, managing symptoms. Understanding these dermatological manifestations is crucial for the timely diagnosis and management of hematological disorders.
色素沉着症是皮肤科诊断的重大挑战,有时反映了潜在的血液病。本综述探讨了血液病中与色素减退相关的临床表现,重点关注白癜风、斑秃和综合白化病。白癜风是一种针对黑色素细胞的自身免疫性疾病,涉及基因多态性与免疫反应之间的相互作用,尤其是CD8+ T细胞和IFN-γ。药物诱发的白癜风,特别是免疫检查点抑制剂和小分子靶向抗癌疗法,凸显了免疫失调的重要性。白斑病是一种炎症性皮肤病,可能预示着血液系统受累,如深部白斑病和放疗后皮损。与影响黑色素生成的各种基因突变有关的综合征性白化病通常会出现血液学异常。治疗方法主要是针对该病症特有的免疫途径,如果无法做到这一点,则需要控制症状。了解这些皮肤病表现对于及时诊断和治疗血液病至关重要。
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引用次数: 0
Blinatumomab in Children with MRD-Positive B-Cell Precursor Acute Lymphoblastic Leukemia: A Report of 11 Cases Blinatumomab治疗MRD阳性B细胞前体急性淋巴细胞白血病患儿:11例报告
IF 0.9 Q4 Medicine Pub Date : 2024-06-03 DOI: 10.3390/hematolrep16020035
Yi-Lun Wang, Tsung-Yen Chang, Yu‐Chuan Wen, Shu-Ho Yang, Yi-Wen Hsiao, Chia-Chi Chiu, Yu-Chieh Chen, Ruei-Shan Hu, Shih-Hsiang Chen, T. Jaing, Chih-Cheng Hsiao
Background/Objectives: Relapsed B-cell acute lymphoblastic leukemia (B-ALL) remains an unresolved matter of concern regarding adverse outcomes. This case study aimed to evaluate the effectiveness of blinatumomab, with or without door lymphocyte infusion (DLI), in treating measurable residual disease (MRD)-positive B-ALL. Methods: All patients who received blinatumomab salvage therapy were included in this study. Eleven patients were included in the study. All patients were evaluated for MRD-negativity. Results: Before starting blinatumomab therapy, seven patients tested positive for MRD, three tested negative, and one had refractory disease. Hematopoietic cell transplantation (HCT) was reserved for five patients with persistent MRD. Six patients became MRD-negative and subsequent HCT was not performed. Only two patients relapsed; one patient died of relapse, and the other one received carfilzomib-based therapy and was MRD-negative thereafter. Nine patients were MRD-negative at a median follow-up of 28 months (15–52 months). Two of three MRD-positive post-transplant patients remained in complete molecular remission after preemptive DLI at the last follow-up date. In the first salvage, blinatumomab may achieve complete remission and bridging to HCT in pediatric patients with end-of-induction MRD-positive B-cell precursor ALL. Conclusions: The decision on how to treat post-transplant relapse continues to affect survival outcomes. Blinatumomab combined with DLI may extend the armamentarium of release options for high-risk pediatric patients. This approach is encouraging for high-risk ALL patients who are MRD-positive post-transplantation.
背景/目标:复发的B细胞急性淋巴细胞白血病(B-ALL)的不良结局仍是一个悬而未决的问题。本病例研究旨在评估blinatumomab联合或不联合门淋巴细胞输注(DLI)治疗可测量残留病(MRD)阳性B-ALL的有效性。治疗方法所有接受过 blinatumomab 挽救疗法的患者均纳入本研究。本研究共纳入 11 例患者。对所有患者进行了MRD阴性评估。研究结果在开始接受 blinatumomab 治疗前,7 例患者的 MRD 检测呈阳性,3 例呈阴性,1 例为难治性疾病。造血细胞移植(HCT)被保留给了5名MRD持续阳性的患者。六名患者的MRD检测结果为阴性,因此没有进行后续的造血干细胞移植。只有两名患者复发,其中一名患者死于复发,另一名患者接受了卡非佐米治疗,此后MRD阴性。九名患者在中位随访 28 个月(15-52 个月)后 MRD 阴性。3名移植后MRD阳性患者中,有2名在接受了先期DLI治疗后,在最后一次随访时仍保持完全分子缓解。在首次挽救治疗中,blinatumomab可使诱导末期MRD阳性B细胞前体ALL儿科患者获得完全缓解并与造血干细胞移植搭桥。结论如何治疗移植后复发仍会影响生存结果。Blinatumomab联合DLI可能会扩大高风险儿科患者的放疗选择范围。对于移植后MRD阳性的高危ALL患者来说,这种方法令人鼓舞。
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引用次数: 0
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Hematology Reports
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