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Clinical and etiological profile of anemia in a clinical hematology department of North Africa : A cross-sectional study 北非临床血液科贫血的临床和病因学概况:一项横断面研究。
IF 0.9 Q4 HEMATOLOGY Pub Date : 2026-01-01 Epub Date: 2026-02-15 DOI: 10.1016/j.lrr.2026.100571
Wiem Boufrikha , Sirine Ben Salem , Nourhene Mazhoud , Arwa Guedich , Nader Slama , Sarra Boukhris , Mohamed Adnene Lattiri
Anemia, a major public health concern impacting physical performance and morbidity, requires etiological assessment as iron deficiency anemia (IDA, 55.7%) may mask hematologic malignancies. This 2022 cross-sectional study at Monastir's Hematology Department included 296 patients (mean age 47 years, female predominance). Anemic syndrome prompted 68% of consultations, with fatigue/dizziness predominant. Mean hemoglobin (Hb) was 7.9 g/dL; severe anemia (Hb <8 g/dL) affected 49.7%. Isolated anemia prevailed (67.6%) over pancytopenia (11.4%). Leading etiologies were IDA (55.7%, mostly idiopathic), megaloblastic anemia (13%, mainly B12 deficiency), Myelodysplastic syndrome (MDS, 11%), multiple myeloma (MM, 5.7%), and acute leukemia (AL, 3.7%). Other causes included renal failure, hypothyroidism, and drug toxicity. The heterogeneous etiological profile underscores the need for systematic, comprehensive diagnostic approaches to identify both benign and malignant causes for optimal management.
贫血是影响身体机能和发病率的主要公共卫生问题,需要进行病因评估,因为缺铁性贫血(IDA, 55.7%)可能掩盖血液学恶性肿瘤。这项2022年在Monastir血液科进行的横断面研究包括296名患者(平均年龄47岁,女性为主)。68%的问诊是贫血综合征引起的,以疲劳/头晕为主。平均血红蛋白(Hb)为7.9 g/dL;重度贫血(Hb)
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引用次数: 0
Delayed fatal neurotoxicity in post CAR-T cell therapy for multiple myeloma, a case report 多发性骨髓瘤CAR-T细胞治疗后的延迟致死性神经毒性,1例报告
IF 0.9 Q4 HEMATOLOGY Pub Date : 2026-01-01 Epub Date: 2026-03-03 DOI: 10.1016/j.lrr.2026.100577
Hamza Khoudari , Abdalla Shoaib , Muhammad Nashatizadeh , Nausheen Ahmed , Forat Lutfi , Muhammad Mushtaq , Leyla Shune , Anurag Singh , Sunil Abhyankar , Joseph McGuirk , Haitham Abdelhakim
Anti-BCMA CAR T-cell therapy, specifically ciltacabtagene autoleucel, has significantly improved outcomes for relapsed/refractory multiple myeloma (RRMM). While early-onset immune-effector cell–associated neurotoxicity syndrome (ICANS) is a recognized complication, delayed-onset and non-ICANS neurological syndromes, such as movement and neurocognitive toxicity (MNT), present unique diagnostic and therapeutic challenges.
We report a case of a 61-year-old female with a 15-year history of RRMM who developed severe, delayed neurotoxicity 50 days after ciltacabtagene autoleucel infusion. The clinical course began with confusion and rapidly progressed to grade 4 ICANS characterized by lethargy, rigidity, and parkinsonian features. Serial MRI imaging revealed evolving, symmetric T2/FLAIR hyperintensities in the basal ganglia and brainstem, and reactive pachymeningitis. Despite aggressive multi modal immunosuppression, the patient’s condition remained refractory and expired on day 93. Post-mortem autopsy confirmed severe bilateral hippocampal sclerosis, diffuse gliosis, and microglial infiltration.
This case highlights a fatal presentation of delayed neurotoxicity that overlaps with the emerging MNT phenotype. As CAR-T therapies expand, this case underscores the necessity for prolonged clinical vigilance and the urgent need for novel management strategies for refractory, late-onset neurotoxicity.
抗bcma CAR - t细胞疗法,特别是ciltacabtagene autoeucel,显著改善了复发/难治性多发性骨髓瘤(RRMM)的预后。虽然早发性免疫效应细胞相关神经毒性综合征(ICANS)是公认的并发症,但迟发性和非ICANS神经综合征,如运动和神经认知毒性(MNT),呈现出独特的诊断和治疗挑战。我们报告一例61岁女性,有15年的RRMM病史,在西他卡他烯自脱醇输注后50天出现严重的迟发性神经毒性。临床病程以混乱开始,并迅速发展为以嗜睡、僵硬和帕金森特征为特征的4级ICANS。连续MRI成像显示基底节区和脑干不断发展,对称的T2/FLAIR高信号,反应性厚性脑膜炎。尽管有积极的多模式免疫抑制,患者的病情仍然难治性,并于93天死亡。死后尸检证实严重的双侧海马硬化,弥漫性胶质细胞增生和小胶质细胞浸润。这个病例强调了与新出现的MNT表型重叠的延迟神经毒性的致命表现。随着CAR-T疗法的扩展,该病例强调了延长临床警惕的必要性,以及迫切需要针对难治性迟发性神经毒性的新管理策略。
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引用次数: 0
Systemic, asymptomatic skeletal muscle infiltration by MALT lymphoma: Defining a 'silent infiltrator' phenotype with 18F-FDG PET/CT MALT淋巴瘤系统性、无症状骨骼肌浸润:18F-FDG PET/CT定义“无症状浸润者”表型
IF 0.9 Q4 HEMATOLOGY Pub Date : 2026-01-01 Epub Date: 2026-02-20 DOI: 10.1016/j.lrr.2026.100573
Yasuyuki Takahashi , Ken Naganuma , Yuka Tanaka , Noriyuki Sakata , Taisuke Kawada , Masahiro Kizaki , Shuji Momose , Morihiro Higashi , Takayuki Tabayashi
We report a rare phenotype of Stage IV extranodal marginal zone lymphoma (MALT lymphoma) manifesting as diffuse, asymptomatic skeletal muscle and bone marrow infiltration in a 60-year-old female. Despite a high tumor burden and intense ¹⁸F-FDG avidity (SUVmax 8.8), muscle enzymes remained normal, indicating a non-destructive "silent infiltrator" growth pattern. The diagnosis was confirmed via biopsy (CD20+, CD5-, CyclinD1-) and negative MYD88 L265P mutation status, excluding lymphoplasmacytic lymphoma. The patient achieved a Complete Metabolic Response following Bendamustine-Rituximab therapy. This case underscores the utility of PET/CT in detecting occult systemic disease and defines a unique, indolent clinical variant of muscular MALT lymphoma.
我们报告一个罕见的IV期结外边缘区淋巴瘤(MALT淋巴瘤)的表型,表现为弥漫性,无症状的骨骼肌和骨髓浸润。尽管高肿瘤负荷和强烈的¹⁸F-FDG依赖性(SUVmax 8.8),肌肉酶仍保持正常,表明非破坏性的“沉默浸润”生长模式。通过活检(CD20+, CD5-, CyclinD1-)和MYD88 L265P阴性突变状态确认诊断,排除淋巴浆细胞性淋巴瘤。患者在苯达莫司汀-利妥昔单抗治疗后获得完全代谢缓解。本病例强调了PET/CT在检测隐蔽性全身性疾病中的应用,并定义了一种独特的、惰性的肌性MALT淋巴瘤临床变体。
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引用次数: 0
Pediatric AML in Sousse, Tunisia: Epidemiologic patterns and hierarchical prognostic factors 在苏塞,突尼斯儿科AML:流行病学模式和分级预后因素
IF 0.9 Q4 HEMATOLOGY Pub Date : 2026-01-01 Epub Date: 2025-12-18 DOI: 10.1016/j.lrr.2025.100556
Donia Bouhoula, Haifa Regaieg, Hela Abroug, Nesrine Ben Sayed, Yossra Ben Youssef
Pediatric acute myeloid leukemia outcomes in resource-limited regions remain understudied, particularly regarding how regional epidemiologic patterns interact with prognostic determinants. Our Tunisian cohort (n=68) reveals distinct pediatric AML features: older presentation (median 11 years), male predominance (63%), and unexpected AML2 subtype prevalence (35%). Cytogenetics showed 24% favorable-risk and 22% adverse-risk cases. Treatment outcomes correlated strongly with risk stratification, emphasizing the need for adapted diagnostic protocols in resource-conscious settings to optimize care pathways.
在资源有限的地区,儿童急性髓性白血病的预后仍未得到充分的研究,特别是关于区域流行病学模式如何与预后决定因素相互作用。我们的突尼斯队列(n=68)揭示了不同的儿科AML特征:年龄较大(中位11岁),男性优势(63%),以及意想不到的AML2亚型患病率(35%)。细胞遗传学显示24%的有利风险和22%的不利风险病例。治疗结果与风险分层密切相关,这强调了在资源意识较强的环境中需要适应诊断方案以优化护理途径。
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引用次数: 0
Meta-analysis of clinical efficacy and safety of decitabine combined with CAG regimen in the treatment of acute myeloid leukemia in the elderly 地西他滨联合CAG方案治疗老年急性髓性白血病的临床疗效和安全性meta分析
IF 0.9 Q4 HEMATOLOGY Pub Date : 2026-01-01 Epub Date: 2025-12-19 DOI: 10.1016/j.lrr.2025.100559
Yanxia He, Lili Zhang, Mengmeng Liu, Fuhua Zhang, Hui Gao

Objective

To systematically evaluate the clinical efficacy and safety of decitabine (DAC) combined with CAG regimen in the treatment of elderly patients with acute myeloid leukemia (AML) using meta-analysis

Methods

The studies reported the clinical efficacy and safety of DAC combined with CAG regimen in the treatment of elderly AML patients were searched in Pubmed, Chinese National Knowledge Infrastructure (CNKI), Wanfang Database, and VIP database by computer. On the basis of the screening criteria, the non-conforming literature was eliminated and the final selected literature was analyzed. The methodological quality was assessed and the research data was extracted. Revman 5.3 software was used to assess the clinical efficacy and safety of DAC combined with CAG in the treatment of elderly AML patients, which were shown with forest map. The funnel plots were used to test publication bias.

Results

Thirteen studies involving 1133 elderly AML patients were included. ‌Meta-analysis demonstrated that‌ in comparison with CAG alone group, the CR rate (Z = 5.50, P < 0.001) and total effective rate (Z = 8.71, P < 0.001) of DAC combined with CAG group were higher, while there was no significant difference in PR rate between the two groups (Z = 1.59, P = 0.11). And the infection rate (Z = 3.56, P < 0.001) and fever rate (Z = 5.86, P < 0.001) of DAC combined with CAG group were increased. There were no significant differences in the rates of hematological adverse reactions (P = 0.14), gastrointestinal reactions (P = 0.05), alopecia (P = 0.39), heart injury (P = 0.55), liver and kidney injury (P = 0.74) and myelosuppression (P = 0.82) between the two groups.

Conclusion

Compared with CAG alone regimen, DAC combined with CAG regimen improves clinical efficacy in elderly AML patients, but increases risks of infection and fever-related adverse events.
目的采用荟萃分析方法系统评价地西他滨(DAC)联合CAG方案治疗老年急性髓性白血病(AML)的临床疗效和安全性。方法通过计算机检索Pubmed、中国知网(CNKI)、万方数据库、VIP数据库中有关DAC联合CAG方案治疗老年AML的临床疗效和安全性的研究。根据筛选标准剔除不符合标准的文献,并对最终入选的文献进行分析。评估方法学质量并提取研究数据。采用Revman 5.3软件评价DAC联合CAG治疗老年AML患者的临床疗效和安全性,并以森林图表示。漏斗图用于检验发表偏倚。结果纳入13项研究,涉及1133例老年AML患者。‌荟萃分析表明,‌与单独CAG组相比,CR率(Z = 5.50,P & lt; 0.001),总有效率(Z = 8.71,P & lt; 0.001)的DAC结合CAG组高,尽管在公关率两组之间无显著差异(Z = 1.59,P = 0.11)。DAC联合CAG组感染率(Z = 3.56,P <; 0.001)和发热率(Z = 5.86,P <; 0.001)升高。没有明显差异的血液不良反应率(P = 0.14),胃肠道反应(P = 0.05),脱发(P = 0.39),心脏损伤(P = 0.55),肝脏和肾脏损伤(P = 0.74)和myelosuppression (P = 0.82)之间的两组。结论与单独CAG方案相比,DAC联合CAG方案可提高老年AML患者的临床疗效,但增加了感染和发热相关不良事件的风险。
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引用次数: 0
Leukocytosis and a JAK2 mutation: The importance of expertise in somatic variant interpretation 白细胞增多症和JAK2突变:体细胞变异解释专业知识的重要性
IF 0.9 Q4 HEMATOLOGY Pub Date : 2026-01-01 Epub Date: 2026-01-13 DOI: 10.1016/j.lrr.2026.100563
Christian J. Puzo , Karl Hager , Michal Rose , Ellice Wong , Christopher A. Tormey , Alexa J. Siddon
This case details a patient presenting with a history of chronic leukocytosis with isolated eosinophilia, who was found to have a JAK2 G571S mutation by a next generation sequencing (NGS) panel for myeloproliferative neoplasm driver mutations. Adjunct NGS testing was performed on a sample of buccal cells to demonstrate this variant was a germline alteration, occurring in the absence of additional disease-causing mutations. The result caused the patient to be re-treated for a prior Strongyloides infection with ivermectin, which resolved his eosinophilia. This patient case highlights the likely benign polymorphic nature of the rare G571S JAK2 mutation that has been previously reported. Moreover, our results stress the importance of appropriate interpretation of rare variants of uncertain significance, namely that clinical decision making should be supported by adjunct genetic testing and with appropriate reference to each patient’s clinical context.
本病例详细介绍了一名慢性白细胞增多症伴孤立嗜酸性粒细胞增多症的患者,通过下一代骨髓增殖性肿瘤驱动突变测序(NGS)小组发现该患者具有JAK2 G571S突变。对口腔细胞样本进行了辅助NGS测试,以证明该变异是在没有其他致病突变的情况下发生的种系改变。结果导致患者再次接受伊维菌素治疗之前的圆形杆菌感染,这解决了他的嗜酸性粒细胞增多。该病例强调了先前报道的罕见的G571S JAK2突变可能的良性多态性性质。此外,我们的研究结果强调了对不确定意义的罕见变异进行适当解释的重要性,即临床决策应该得到辅助基因检测的支持,并适当参考每位患者的临床情况。
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引用次数: 0
The synergistic tumor suppressor effect of CDK4/6 inhibitors and BRD4 inhibitors in acute myeloid leukemia CDK4/6抑制剂和BRD4抑制剂在急性髓性白血病中的协同抑瘤作用
IF 0.9 Q4 HEMATOLOGY Pub Date : 2026-01-01 Epub Date: 2025-12-20 DOI: 10.1016/j.lrr.2025.100558
Kaiqiong Liao , Chong Guo , Min Zhang , Xiaobin Lv , Jinhua Yan

Objective

The objective of this research is to explore the anti-leukemic properties of CDK4/6 inhibitors when used alongside BET inhibitors for treating acute myeloid leukemia (AML), as well as to clarify the molecular mechanisms involved.

Methods

Cell viability was assessed using the CCK-8 assay following treatment of AML cells with varying doses of SHR6390, a CDK4/6 inhibitor, and OTX015, a BET inhibitor.The time- and dose-dependent inhibitory effects of these two drugs on AML cells were assessed, and the respective IC50 and combination index (CI) values after co-treatment were calculated. The effects of SHR6390 and OTX015 on the growth potential of AML cells were additionally examined through soft agar colony formation assays and flow cytometry. Furthermore, RNA sequencing and Western blot analysis were conducted on cells treated with both drugs. The aim of this study is to explore the mechanism by which SHR6390 and OTX015 synergistically inhibit the proliferation of AML cells.The anti-tumor activity of SHR6390 and/or OTX015 in AML xenograft mice was also investigated through animal experiments.

Results

1. Either the CDK4/6 inhibitor SHR6390 or the BRD4 inhibitor OTX015, or a combination of the two, were employed to hinder both the survival and proliferation of cell lines associated with acute myeloid leukemia, showing a synergistic effect. 2. The combined application of SHR6390 and OTX015 markedly suppresses the invasive and migratory capacities of acute myeloid leukemia cells.3. The use of both SHR6390 and OTX015 induces apoptosis in acute myeloid leukemia cells while also disrupting cell cycle progression, leading to a halt before DNA replication occurs. 4. SHR6390 and OTX015 hinder the proliferation of acute myeloid leukemia cells by targeting both the PI3K-AKT-mTOR and the Wnt-β-Catenin pathway. 5. SHR6390 and OTX015 Synergistically Inhibit the Growth of AML Xenografts In Vivo.
本研究的目的是探讨CDK4/6抑制剂与BET抑制剂联合治疗急性髓性白血病(AML)时的抗白血病特性,并阐明其中的分子机制。方法用不同剂量的SHR6390(一种CDK4/6抑制剂)和OTX015(一种BET抑制剂)治疗AML细胞后,使用CCK-8法评估细胞活力。评估两种药物对AML细胞的抑制作用的时间依赖性和剂量依赖性,并计算两种药物联合治疗后各自的IC50和联合指数(CI)值。另外,通过软琼脂集落形成实验和流式细胞术检测SHR6390和OTX015对AML细胞生长潜能的影响。此外,对两种药物处理的细胞进行RNA测序和Western blot分析。本研究旨在探讨SHR6390和OTX015协同抑制AML细胞增殖的机制。通过动物实验研究了SHR6390和/或OTX015在AML异种移植小鼠中的抗肿瘤活性。利用CDK4/6抑制剂SHR6390或BRD4抑制剂OTX015,或两者联合抑制急性髓系白血病相关细胞系的存活和增殖,显示出协同效应。2. SHR6390和OTX015联合应用可显著抑制急性髓系白血病细胞的侵袭和迁移能力。使用SHR6390和OTX015均可诱导急性髓系白血病细胞凋亡,同时也会破坏细胞周期进程,导致DNA复制发生前停止。4. SHR6390和OTX015通过靶向PI3K-AKT-mTOR和Wnt-β-Catenin通路抑制急性髓系白血病细胞的增殖。5. SHR6390和OTX015在体内协同抑制AML异种移植物生长
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引用次数: 0
Richter-like transformation of CLL/SLL after a temporary hold of ibrutinib: A case report 暂时使用依鲁替尼后CLL/SLL的richter样转化:1例报告
IF 0.9 Q4 HEMATOLOGY Pub Date : 2026-01-01 Epub Date: 2026-03-02 DOI: 10.1016/j.lrr.2026.100578
Tung-Lin Chiang , John Frater , Amanda Cashen
Bruton tyrosine kinase (BTK) inhibitors are used to treat chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL), providing survival benefits over traditional chemoimmunotherapy. Richter transformation is the transformation of CLL/SLL into an aggressive lymphoma, typically necessitating an immediate start of treatment. This case report presents a CLL/SLL patient developing pathology-proven Richter transformation after a short hold of the BTK inhibitor, without clinical evidence of disease progression. This case also demonstrates that the pathology-only Richter transformation after a pause of the BTK inhibitor can respond to resumption of the inhibitor, without the addition of intensive chemotherapy.
布鲁顿酪氨酸激酶(BTK)抑制剂被用于治疗慢性淋巴细胞白血病/小淋巴细胞淋巴瘤(CLL/SLL),比传统的化学免疫疗法提供生存优势。里希特转化是CLL/SLL向侵袭性淋巴瘤的转化,通常需要立即开始治疗。本病例报告介绍了一名CLL/SLL患者在短期服用BTK抑制剂后发生病理证实的Richter转化,没有疾病进展的临床证据。该病例还表明,暂停BTK抑制剂后病理上的Richter转化可以在不增加强化化疗的情况下对抑制剂的恢复有反应。
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引用次数: 0
Therapeutic outcomes of acute promyelocytic leukemia in children 儿童急性早幼粒细胞白血病的治疗效果
IF 0.9 Q4 HEMATOLOGY Pub Date : 2026-01-01 Epub Date: 2026-02-15 DOI: 10.1016/j.lrr.2026.100569
Sirine Chatti, Emna Azza, Marwa Bahri, Roua Hsasna, Yosr Ben Abdennebi, Lamia Aissaoui

Introduction

In children, acute promyelocytic leukemia is a distinct and underreported entity. The objective of our study is to report the therapeutic outcomes of APL in the pediatric population.

Methods

This was a descriptive, retrospective, single-center study including 30 patients diagnosed with APL over an 18-year period. Patients were treated in the Hematology Department of Aziza Othmana Hospital in Tunis.

Results

The median age at diagnosis was 11 years, with a sex ratio of 0.87. Half of the patients were classified as high-risk. Post-induction cytologic remission was achieved in 86.6 % of patients. There were five cases of relapse, 60 % of which were early relapses. The 5-year overall survival rate was 70.6 %.The 5-year event-free survival rate was 72 %. The 5-year relapse-free survival rate was 80 %.

Conclusion

Although our results were satisfactory, they revealed a poor prognosis for relapsed patients. A larger study focusing on relapse characteristics is needed.
在儿童中,急性早幼粒细胞白血病是一种独特且未被报道的疾病。我们研究的目的是报告小儿APL的治疗结果。方法:本研究是一项描述性、回顾性、单中心研究,纳入了30例APL患者,随访时间超过18年。患者在突尼斯Aziza Othmana医院血液科接受治疗。结果诊断年龄中位数为11岁,性别比为0.87。一半的患者被归为高危人群。86.6%的患者诱导后细胞学缓解。复发5例,早期复发占60%。5年总生存率为70.6%。5年无事件生存率为72%。5年无复发生存率为80%。结论虽然我们的结果是令人满意的,但它们显示复发患者的预后较差。需要对复发特征进行更大规模的研究。
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引用次数: 0
Imatinib-induced rhabdomyolysis: A case report 伊马替尼诱导横纹肌溶解1例
IF 0.9 Q4 HEMATOLOGY Pub Date : 2026-01-01 Epub Date: 2026-01-12 DOI: 10.1016/j.lrr.2026.100561
Jordan Rubenstein, Sarah M. Schwartz, Jay Vankawala, Michael Caplan, Steven Shea, Joseph G. Jurcic
Imatinib, a tyrosine kinase inhibitor used for chronic myeloid leukemia (CML) and gastrointestinal stromal tumors (GIST), is associated with myalgia and creatine kinase (CK) elevations, though severe rhabdomyolysis and myopathy are rare. We report an 83-year-old woman with CML who developed progressive proximal weakness, dark urine, and acute kidney injury after three years of imatinib therapy. Laboratory evaluation revealed CK >24,000 U/L, transaminitis, and myoglobinuria. MRI showed diffuse muscle and fascial edema, while autoimmune testing was negative. Imatinib and rosuvastatin were discontinued, and the patient was managed with intravenous fluids and supportive care. CK and renal function normalized within 10 days, with substantial recovery of strength. The strong temporal relationship between drug withdrawal and improvement implicates imatinib as the etiology. This case represents one of the most severe reported instances of imatinib-induced rhabdomyolysis. Early recognition and discontinuation are essential to prevent life-threatening sequelae.
伊马替尼是一种酪氨酸激酶抑制剂,用于慢性髓性白血病(CML)和胃肠道间质瘤(GIST),与肌痛和肌酸激酶(CK)升高有关,尽管严重的横纹肌溶解和肌病很少见。我们报告一位83岁的CML女性患者,在伊马替尼治疗三年后出现进行性近端无力、尿色深和急性肾损伤。实验室检查显示CK >; 24000 U/L,转氨炎和肌红蛋白尿。MRI显示弥漫性肌肉和筋膜水肿,自身免疫检测为阴性。停用伊马替尼和瑞舒伐他汀,患者接受静脉输液和支持性护理。10天内CK和肾功能恢复正常,体力恢复明显。停药与改善之间的时间关系暗示伊马替尼可能是病因。本病例是伊马替尼引起的横纹肌溶解最严重的病例之一。早期识别和停止治疗对于预防危及生命的后遗症至关重要。
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引用次数: 0
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Leukemia Research Reports
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