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IF 0.9 Q4 HEMATOLOGY Pub Date : 2025-01-01
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引用次数: 0
IF 0.9 Q4 HEMATOLOGY Pub Date : 2025-01-01
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引用次数: 0
IF 0.9 Q4 HEMATOLOGY Pub Date : 2025-01-01
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引用次数: 0
Pediatric chronic myeloid leukemia: A decade of clinical experience at the NBK specialized hospital for children 儿童慢性髓性白血病:在NBK儿童专科医院的十年临床经验
IF 0.9 Q4 HEMATOLOGY Pub Date : 2025-01-01 DOI: 10.1016/j.lrr.2025.100549
Maha Bourusly , Mohammad Adil Obaid , Nashwa Farag , Mona Bourhama , Sundos Alsharidah , Eman Almatar
Pediatric chronic myeloid leukemia is rare and differs from adult disease. We retrospectively reviewed 12 children (median age 8.4 years, six males) treated with imatinib (340 mg/m²/day) from 2010 to 2020; dasatinib was used for intolerance. All presented with leukocytosis (median WBC, 358 × 109/L) and splenomegaly. Imatinib achieved complete hematologic response in 92% by 3 months and 100% by 6 months; major molecular response was observed in all evaluable cases at 12 months. Three who lost response switched to dasatinib and maintained remission; two attained treatment-free remission. TKIs are effective; prospective studies should optimize risk stratification and discontinuation.
小儿慢性髓性白血病是罕见的,不同于成人疾病。我们回顾性分析了2010年至2020年接受伊马替尼(340 mg/m²/天)治疗的12名儿童(中位年龄8.4岁,6名男性);达沙替尼用于治疗不耐受。所有患者均表现为白细胞增多(白细胞中位数为358 × 109/L)和脾肿大。伊马替尼在3个月和6个月达到了92%和100%的完全血液学缓解;在12个月时,所有可评估的病例均观察到主要的分子反应。3名失去反应的患者改用达沙替尼并维持缓解;两名患者获得了无治疗缓解。tki是有效的;前瞻性研究应优化风险分层和停药。
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引用次数: 0
Corrigendum to “First-line therapy with daratumumab, lenalidomide and dexamethasone for patient with POEMS syndrome: A case report” [Leukemia Research Reports (2024) Volume 22, 100491] “用达拉单抗、来那度胺和地塞米松治疗POEMS综合征患者的一线治疗:一个病例报告”的勘误表[白血病研究报告(2024)卷22,100491]
IF 0.7 Q4 HEMATOLOGY Pub Date : 2025-01-01 DOI: 10.1016/j.lrr.2025.100499
E. Amabile, F. Fazio, M. Martelli, M.T. Petrucci
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引用次数: 0
Catastrophic asparaginase-induced cerebral and systemic thrombosis in a young female with T-cell lymphoblastic lymphoma: A case report & literature review 灾难性天冬酰胺酶诱发的年轻女性t细胞淋巴母细胞淋巴瘤脑及全身血栓:1例报告及文献复习
IF 0.7 Q4 HEMATOLOGY Pub Date : 2025-01-01 DOI: 10.1016/j.lrr.2025.100526
Shihab Ahmed Ibrahim Ahmed, Amr Suliman AlHanbali
Asparaginase is a cornerstone in the treatment of acute lymphoblastic leukemia (ALL) and its variant, T-cell lymphoblastic lymphoma (T-LBL), particularly in adolescents and young adults (AYA). However, its use is associated with a significant risk of thrombotic complications due to its profound effects on coagulation pathways. We report a catastrophic case of asparaginase-induced cerebral and systemic thrombosis in a previously healthy 20-year-old female with T-LBL. Despite prophylactic anticoagulation, the patient developed extensive cerebral venous sinus thrombosis, deep vein thrombosis, and pulmonary embolism, necessitating mechanical thrombectomy, Argatroban therapy, and long-term anticoagulation. This case underscores the multifactorial pathophysiology of asparaginase-associated thrombosis and highlights the need for individualized risk assessment, vigilant monitoring, and dynamic anticoagulation strategies. A comprehensive literature review is provided to contextualize the epidemiology, mechanisms, risk factors, prevention, and management of this life-threatening complication, with practical recommendations for optimizing care in high-risk patients.
天冬酰胺酶是治疗急性淋巴细胞白血病(ALL)及其变种t细胞淋巴母细胞淋巴瘤(T-LBL)的基石,特别是在青少年和年轻人(AYA)中。然而,由于其对凝血途径的深远影响,其使用与血栓并发症的显著风险相关。我们报告一个灾难性的病例天冬酰胺酶诱导的脑和全身血栓形成在一个以前健康的20岁女性与T-LBL。尽管进行了预防性抗凝治疗,但患者出现了广泛的脑静脉窦血栓、深静脉血栓和肺栓塞,需要机械取栓、阿加曲班治疗和长期抗凝。该病例强调了天冬酰胺酶相关血栓形成的多因素病理生理学,并强调了个体化风险评估、警惕监测和动态抗凝策略的必要性。本文对这一危及生命的并发症的流行病学、机制、危险因素、预防和管理进行了全面的文献综述,并提出了优化高危患者护理的实用建议。
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引用次数: 0
Analysis of atypical clinical manifestations in eight patients with AIDS complicated by lymphoma 艾滋病合并淋巴瘤8例不典型临床表现分析
IF 0.9 Q4 HEMATOLOGY Pub Date : 2025-01-01 DOI: 10.1016/j.lrr.2025.100540
Peng fei Tao, Jincheng Lou, Xicheng Wang

Objective

Acquired Immunodeficiency Syndrome (AIDS)-related lymphoma has diverse clinical manifestations, and its diagnosis is often challenging. Misdiagnosis can delay treatment and affect patient prognosis. We assessed the clinical data of eight patients with atypical clinical manifestations of AIDS-related lymphoma, to enhance physicians' understanding of these patients, and reduce the potential for misdiagnosis.

Methods

A retrospective analysis was conducted on eight patients with atypical manifestations of AIDS-related lymphoma admitted to the Department of Infectious Diseases of Yunnan Provincial Hospital between May 2017 and May 2023. They were initially misdiagnosed with opportunistic infections, and were later diagnosed with lymphoma.

Results

The patients comprised five males and three females. Cluster of Differentiation 4 (CD4) counts were lower than 200/μl for all patients, and inflammatory marker levels were elevated to varying degrees. Four patients had recurrent fever, one had bleeding, one had pulmonary infection, one had long-term diarrhoea, and one had visual impairment as the primary symptoms. Six patients were diagnosed through bone marrow cytology and biopsy, one through colonoscopy and pathological biopsy, and one through computed tomography-guided percutaneous lung biopsy. All patients had extranodal involvement, including one case in the intestine, one in the lung, and six in the bone marrow. All patients were at lymphoma stage IV, with four in Group A and four in group B.

Conclusion

In patients with AIDS, particularly those with low CD4 counts and unexplained fever, atypical lymphoma should be considered, and tissue biopsy should be performed to further confirm the diagnosis.
目的获得性免疫缺陷综合征(AIDS)相关淋巴瘤具有多种临床表现,其诊断往往具有挑战性。误诊会延误治疗,影响患者预后。我们对8例临床表现不典型的艾滋病相关淋巴瘤患者的临床资料进行分析,以提高医生对这些患者的认识,减少误诊的可能性。方法对2017年5月至2023年5月云南省医院感染性疾病科收治的8例非典型表现艾滋病相关淋巴瘤患者进行回顾性分析。他们最初被误诊为机会性感染,后来被诊断为淋巴瘤。结果男性5例,女性3例。所有患者CD4细胞计数均低于200/μl,炎症标志物水平均不同程度升高。4例反复发热,1例出血,1例肺部感染,1例长期腹泻,1例以视力受损为主要症状。6例患者通过骨髓细胞学和活检诊断,1例通过结肠镜检查和病理活检诊断,1例通过计算机断层扫描引导下的经皮肺活检诊断。所有患者均有结外受累,包括1例肠、1例肺和6例骨髓。所有患者均为淋巴瘤IV期,其中A组4例,b组4例。结论艾滋病患者,特别是CD4计数低、不明原因发热者,应考虑非典型淋巴瘤,并行组织活检进一步确诊。
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引用次数: 0
Venetoclax combination with Cladribine, idarubicin, Cytarabine for relapsed T-Cell acute lymphoblastic leukemia/lymphoblastic lymphoma treatment: A case report and literature review Venetoclax联合克拉德滨、依达柔比星、阿糖胞苷治疗复发性t细胞急性淋巴细胞白血病/淋巴细胞淋巴瘤1例报告并文献复习
IF 0.7 Q4 HEMATOLOGY Pub Date : 2025-01-01 DOI: 10.1016/j.lrr.2025.100506
Alaa Eldein Yahia , Ibrahim Motabi , Abdullah A. Alsakkaf , Kamal Alzahrani , Laila M. Alsuhaibani , Bilal Albtoosh , Abdullah Khaled AlBathi , Abdullah M. Alrajhi
Acute lymphoblastic leukemia (ALL) represents only 20 % of adult acute leukemias, while Lymphoblastic lymphoma is even rarer, accounting for 2 % of adult non-Hodgkin lymphomas. T-acute lymphoblastic leukemia (T-ALL) and T-lymphoblastic lymphoma (T-LBL) are neoplasms characterized by the presence of immature T-cell precursors or lymphoblasts. Relapsed T-ALL or LBL is associated with a very poor prognosis, necessitating the exploration of novel therapeutic approaches. This case report describes the use of Venetoclax in combination with Cladribine, Idarubicin, and Cytarabine (CLIA) as salvage therapy for relapsed T-ALL/T-LBL. The treatment regimen resulted in remission and negative minimal residual disease. However, it was accompanied by delayed count recovery, febrile neutropenia, and Central Line-Associated Bloodstream Infection. The management of central nervous system involvement was challenging due to low platelet counts requiring transfusion support. The findings highlight the need for further investigation into the efficacy and optimal therapeutic regimen for relapsed T-ALL/T-LBL. Additionally, the case emphasizes the importance of early salvage therapy and potentially consolidative hematopoietic stem cell transplantation for improved survival outcomes in relapsed T-ALL/T-LBL patients.
急性淋巴母细胞白血病(ALL)仅占成人急性白血病的20%,而淋巴母细胞淋巴瘤更罕见,占成人非霍奇金淋巴瘤的2%。t急性淋巴母细胞白血病(T-ALL)和t淋巴母细胞淋巴瘤(T-LBL)是一种以未成熟t细胞前体或淋巴母细胞存在为特征的肿瘤。复发的T-ALL或LBL预后非常差,需要探索新的治疗方法。本病例报告描述了使用Venetoclax联合cladriine, Idarubicin和Cytarabine (CLIA)作为复发T-ALL/T-LBL的补救性治疗。治疗方案导致缓解和阴性最小残留疾病。然而,它伴随着计数恢复延迟、发热性中性粒细胞减少和中央线相关血流感染。由于血小板计数低,需要输血支持,中枢神经系统受累的管理具有挑战性。研究结果强调需要进一步研究复发T-ALL/T-LBL的疗效和最佳治疗方案。此外,该病例强调了早期补救性治疗和潜在的巩固性造血干细胞移植对改善复发T-ALL/T-LBL患者生存结果的重要性。
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引用次数: 0
AL amyloidosis with elevated peripheral blood cell counts – A frequent association with liver involvement. A single-center retrospective study AL淀粉样变伴外周血细胞计数升高-常与肝脏受累有关。单中心回顾性研究
IF 0.7 Q4 HEMATOLOGY Pub Date : 2025-01-01 DOI: 10.1016/j.lrr.2025.100509
Mateusz Ziarkiewicz , Justyna Szczygieł , Marta Legatowicz-Koprowska , Joanna Drozd-Sokołowska , Piotr Boguradzki , Krzysztof Jamroziak , Grzegorz Basak

Background

AL amyloidosis is a systemic protein misfolding disorder characterized by organ deposition of monoclonal immunoglobulin fragments, with insidious onset and progressive course. The plasma cell clone in the bone marrow is relatively small and typically does not impair hematopoiesis, in contrast to multiple myeloma. Herein we present a novel observation of increased thrombocyte, leukocyte and erythrocyte counts in a subset of AL amyloidosis patients.

Material and Methods

We performed a retrospective analysis of medical records of all consecutive patients diagnosed with AL amyloidosis at the Medical University of Warsaw in years 2001–2022, which included clinical, pathological and laboratory data, as well as treatment protocols and outcomes.

Results

Twenty-three patients out of 124 (18.4 %) included had elevated blood counts: 17 (13.6 %) had leukocytosis with neutrophilia, 7 (5.6 %) had thrombocytosis, whereas 2 (1.6 %) had erythrocytosis. In comparison to the remaining AL population this subgroup was characterized by younger age (median 57 vs 62 years, p = 0.018), higher frequency of hepatomegaly (42.9 % vs.14.7 %, p = 0.004), higher median alkaline phosphatase concentration (129 U/L vs 93 U/L, p = 0.006) and more frequent hepatic amyloidosis (34.8 % vs 10.3 %, p = 0.003). None of the patients had definite features of a myeloproliferative neoplasm, although genetic testing was available in 5 out of 9 cases with thrombocytosis or erythrocytosis. There were no significant differences in terms of survival between patients with elevated cell counts and non-polycythemic patients (median overall survival 2.9 vs 6.6 years, p = 0.51, median event-free survival 0.7 vs 1.8 years, p = 0.29, respectively).

Conclusions

Elevated peripheral blood counts in a subset of patients with AL amyloidosis constitute a rare but significant phenomenon and appear to be associated with frequent hepatic involvement. We hypothesize that cytokine deregulation and hyposplenism may belong to its pathomechanisms.
背景:淀粉样变性是一种以单克隆免疫球蛋白片段器官沉积为特征的全身性蛋白质错误折叠疾病,发病隐匿,病程渐进。与多发性骨髓瘤相比,骨髓中的浆细胞克隆相对较小,通常不会损害造血功能。在这里,我们提出了一个新的观察增加的血小板,白细胞和红细胞计数在一个子集AL淀粉样变性患者。材料和方法我们对2001-2022年在华沙医科大学诊断为AL淀粉样变性的所有连续患者的医疗记录进行了回顾性分析,包括临床、病理和实验室数据,以及治疗方案和结果。结果124例患者中23例(18.4%)出现血球计数增高,其中白细胞增多伴中性粒细胞增多17例(13.6%),血小板增多7例(5.6%),红细胞增多2例(1.6%)。与其他AL人群相比,该亚组的特点是年龄更年轻(中位57岁对62岁,p = 0.018),肝肥大发生率更高(42.9%对14.7%,p = 0.004),碱性磷酸酶中位浓度更高(129 U/L对93 U/L, p = 0.006),肝淀粉样变发生率更高(34.8%对10.3%,p = 0.003)。虽然9例有血小板增多或红细胞增多的患者中有5例进行了基因检测,但没有一例患者具有骨髓增生性肿瘤的明确特征。细胞计数升高的患者和非红细胞增多症患者的生存期没有显著差异(中位总生存期为2.9年vs 6.6年,p = 0.51,中位无事件生存期为0.7年vs 1.8年,p = 0.29)。结论:AL淀粉样变患者的外周血计数升高是一种罕见但重要的现象,似乎与频繁的肝脏受累有关。我们推测细胞因子失调和脾功能减退可能属于其病理机制。
{"title":"AL amyloidosis with elevated peripheral blood cell counts – A frequent association with liver involvement. A single-center retrospective study","authors":"Mateusz Ziarkiewicz ,&nbsp;Justyna Szczygieł ,&nbsp;Marta Legatowicz-Koprowska ,&nbsp;Joanna Drozd-Sokołowska ,&nbsp;Piotr Boguradzki ,&nbsp;Krzysztof Jamroziak ,&nbsp;Grzegorz Basak","doi":"10.1016/j.lrr.2025.100509","DOIUrl":"10.1016/j.lrr.2025.100509","url":null,"abstract":"<div><h3>Background</h3><div>AL amyloidosis is a systemic protein misfolding disorder characterized by organ deposition of monoclonal immunoglobulin fragments, with insidious onset and progressive course. The plasma cell clone in the bone marrow is relatively small and typically does not impair hematopoiesis, in contrast to multiple myeloma. Herein we present a novel observation of increased thrombocyte, leukocyte and erythrocyte counts in a subset of AL amyloidosis patients.</div></div><div><h3>Material and Methods</h3><div>We performed a retrospective analysis of medical records of all consecutive patients diagnosed with AL amyloidosis at the Medical University of Warsaw in years 2001–2022, which included clinical, pathological and laboratory data, as well as treatment protocols and outcomes.</div></div><div><h3>Results</h3><div>Twenty-three patients out of 124 (18.4 %) included had elevated blood counts: 17 (13.6 %) had leukocytosis with neutrophilia, 7 (5.6 %) had thrombocytosis, whereas 2 (1.6 %) had erythrocytosis. In comparison to the remaining AL population this subgroup was characterized by younger age (median 57 vs 62 years, <em>p</em> = 0.018), higher frequency of hepatomegaly (42.9 % vs.14.7 %, <em>p</em> = 0.004), higher median alkaline phosphatase concentration (129 U/L vs 93 U/L, <em>p</em> = 0.006) and more frequent hepatic amyloidosis (34.8 % vs 10.3 %, <em>p</em> = 0.003). None of the patients had definite features of a myeloproliferative neoplasm, although genetic testing was available in 5 out of 9 cases with thrombocytosis or erythrocytosis. There were no significant differences in terms of survival between patients with elevated cell counts and non-polycythemic patients (median overall survival 2.9 vs 6.6 years, <em>p</em> = 0.51, median event-free survival 0.7 vs 1.8 years, <em>p</em> = 0.29, respectively).</div></div><div><h3>Conclusions</h3><div>Elevated peripheral blood counts in a subset of patients with AL amyloidosis constitute a rare but significant phenomenon and appear to be associated with frequent hepatic involvement. We hypothesize that cytokine deregulation and hyposplenism may belong to its pathomechanisms.</div></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"23 ","pages":"Article 100509"},"PeriodicalIF":0.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143843498","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Retrospective claims data analysis of ASCT characteristics and costs for working-age, multiple myeloma patients in the US, 2017–2019 2017-2019年美国工龄多发性骨髓瘤患者ASCT特征和费用的回顾性索赔数据分析。
IF 0.7 Q4 HEMATOLOGY Pub Date : 2025-01-01 DOI: 10.1016/j.lrr.2024.100496
Whanhui Chi , Juhyeon Song , Tyler J. Varisco
Multiple myeloma (MM) is a rare hematologic malignancy with a 5-year survival rate of 52 %. For transplant-eligible MM patients, high-dose chemotherapy followed by autologous stem cell transplant (ASCT) is recommended. Given the complexities of the ASCT procedure, understanding patient-specific factors and their impact on treatment decisions is essential.
Our study examines patient characteristics and patterns of health resource utilization associated with ASCT receipt in patients with MM.
This retrospective study used the Merative™ MarketScan® database from 2017 to 2019 to analyze working-aged adults (18–65 years) with MM. We categorized 643 ASCT recipients by demographic characteristics (age, sex, region, employment status, year of ASCT procedure) and clinical factors (Charlson Comorbidity Index score). We assessed health resource utilization, focusing on ASCT-related costs, including total payments and hospitalization duration. Descriptive statistics were calculated for all variables, with means, medians, standard deviations for continuous variables, and frequencies for categorical variables. Pearson correlation assessed the relationship between total payment and hospitalization duration.
Over 80 % of patients were over 50, highlighting the need for age-specific clinical strategies. Most patients had CCI scores of 2–4, indicating a moderate comorbidity burden. The mean hospitalization duration was 21.71 days, with average ASCT costs totaling $166,235.99. The correlation coefficient of 0.21 indicated that total payments also increase as the number of hospitalization days increases.
These findings highlight the need for tailored care approaches and resource allocation in ASCT, informing future research and clinical decision-making.
多发性骨髓瘤(MM)是一种罕见的血液系统恶性肿瘤,5年生存率为52%。对于符合移植条件的MM患者,推荐高剂量化疗后进行自体干细胞移植(ASCT)。鉴于ASCT程序的复杂性,了解患者特异性因素及其对治疗决策的影响至关重要。我们的研究检查了MM患者接受ASCT相关的患者特征和健康资源利用模式。这项回顾性研究使用了2017年至2019年Merative™MarketScan®数据库,分析了患有MM的工作年龄成年人(18-65岁)。我们根据人口统计学特征(年龄、性别、地区、就业状况、ASCT手术年份)和临床因素(Charlson共病指数评分)对643名ASCT接受者进行了分类。我们评估了卫生资源利用情况,重点关注与asct相关的费用,包括总费用和住院时间。对所有变量进行描述性统计,包括连续变量的均值、中位数、标准差和分类变量的频率。Pearson相关性评估总付款额与住院时间的关系。超过80%的患者超过50岁,这突出了针对年龄的临床策略的必要性。大多数患者的CCI评分为2-4,表明有中度合并症负担。平均住院时间为21.71天,平均ASCT费用总计为166,235.99美元。相关系数为0.21,表明随着住院天数的增加,支付总额也随之增加。这些发现强调了在ASCT中定制护理方法和资源分配的必要性,为未来的研究和临床决策提供了信息。
{"title":"Retrospective claims data analysis of ASCT characteristics and costs for working-age, multiple myeloma patients in the US, 2017–2019","authors":"Whanhui Chi ,&nbsp;Juhyeon Song ,&nbsp;Tyler J. Varisco","doi":"10.1016/j.lrr.2024.100496","DOIUrl":"10.1016/j.lrr.2024.100496","url":null,"abstract":"<div><div>Multiple myeloma (MM) is a rare hematologic malignancy with a 5-year survival rate of 52 %. For transplant-eligible MM patients, high-dose chemotherapy followed by autologous stem cell transplant (ASCT) is recommended. Given the complexities of the ASCT procedure, understanding patient-specific factors and their impact on treatment decisions is essential.</div><div>Our study examines patient characteristics and patterns of health resource utilization associated with ASCT receipt in patients with MM.</div><div>This retrospective study used the Merative™ MarketScan® database from 2017 to 2019 to analyze working-aged adults (18–65 years) with MM. We categorized 643 ASCT recipients by demographic characteristics (age, sex, region, employment status, year of ASCT procedure) and clinical factors (Charlson Comorbidity Index score). We assessed health resource utilization, focusing on ASCT-related costs, including total payments and hospitalization duration. Descriptive statistics were calculated for all variables, with means, medians, standard deviations for continuous variables, and frequencies for categorical variables. Pearson correlation assessed the relationship between total payment and hospitalization duration.</div><div>Over 80 % of patients were over 50, highlighting the need for age-specific clinical strategies. Most patients had CCI scores of 2–4, indicating a moderate comorbidity burden. The mean hospitalization duration was 21.71 days, with average ASCT costs totaling $166,235.99. The correlation coefficient of 0.21 indicated that total payments also increase as the number of hospitalization days increases.</div><div>These findings highlight the need for tailored care approaches and resource allocation in ASCT, informing future research and clinical decision-making.</div></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"23 ","pages":"Article 100496"},"PeriodicalIF":0.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11730578/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142984835","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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Leukemia Research Reports
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