• 文章类型: Journal Article
    目的/背景乳腺白血病(BL)是一种罕见的乳腺恶性肿瘤,其治疗方法与其他恶性肿瘤不同。然而,它很容易与其他条件混淆;因此,如何准确诊断至关重要。我们回顾性分析了13例患者的影像学表现,以提供诊断参考。方法回顾性分析2015年1月至2023年4月在北京大学人民医院行影像学检查的13例经活检证实的BL患者的临床资料。通过超声(US)获得的成像结果,乳房X线摄影(MMG),磁共振成像(MRI),和正电子发射断层扫描/计算机断层扫描(PET/CT)进行了分析,并比较了这些方法诊断BL的检出率。结果13例患者共检出29个病灶。这些患者在白血病治疗后几个月出现明显的肿块或乳房肿胀,主要涉及双侧乳房。对13例患者进行了超声检查,并检测到所有病变。大多数已确定的肿块是低回声的,边界不清,不规则形状,后回声没有增强,没有充足的血液流动。对五名患者进行了MMG,露出的乳房肿块,建筑扭曲,也没有异常.对四名患者进行了MRI检查,并检测到所有病变;大多数病变在T1加权成像上为低信号,在T2加权成像和弥散加权成像上为高强度,具有降低的表观扩散系数和不均匀增强。增强曲线主要为流入模式。4例患者行PET/CT检查,2例患者出现代谢亢进,另外两个没有明显的放射性吸收。结论与MMG和PET/CT相比,US和MRI具有较高的检出率。此外,与MRI相比,美国便宜,方便高效;因此,应该是诊断BL的首选.
    Aims/Background Breast leukaemia (BL) is a rare breast malignancy that is treated differently from other malignant conditions. However, it is easily confused with other conditions; therefore, how to accurately diagnose is crucial. We retrospectively analysed the imaging findings of 13 patients to provide a diagnostic reference. Methods From January 2015 to April 2023, 13 patients with BL confirmed by biopsy who underwent imaging in Peking University People\'s hospital were retrospectively analysed. The imaging findings obtained via ultrasound (US), mammography (MMG), magnetic resonance imaging (MRI), and positron emission tomography/computed tomography (PET/CT) were analysed, and the detection rates of these methods for diagnosing BL were compared. Results Twenty-nine lesions were detected in the 13 patients. These patients presented with palpable masses or breast swelling several months after treatment for leukaemia, mainly involving the bilateral breasts. Ultrasonography was performed for 13 patients, and all lesions were detected. Most of the identified masses were hypoechoic and had indistinct boundaries, irregular shapes, no enhancement of the posterior echo, and no abundant blood flow. MMG was performed for five patients, revealing breast masses, architectural distortion, and no abnormalities. MRI was performed for four patients, and all lesions were detected; most of the lesions were hypointense on T1-weighted imaging and hyperintense on T2-weighted imaging and diffusion-weighted imaging, with a decreased apparent diffusion coefficient and inhomogeneous enhancement. The enhancement curves were mostly inflow patterns. PET/CT was performed for four patients; two patients had hypermetabolism, and the other two had no obvious radioactive uptake. Conclusion Compared to MMG and PET/CT, US and MRI have higher detection rates. Furthermore, compared to MRI, US is inexpensive, convenient and efficient; therefore, it should be the first choice for diagnosing BL.
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  • 文章类型: Case Reports
    本研究旨在阐明最初表现为心脏压塞的小儿急性髓细胞性白血病(AML)的临床特征,并分享治疗经验。
    五名儿科患者最初被诊断为AML并伴有心脏髓样肉瘤(MS)。诊断是通过检查我们的医院记录并回顾1990年至2023年7月的相关文献来建立的,可通过MEDLINE/PubMed访问。我们全面评估了这些患者的临床特征和治疗方式。
    5名儿科患者出现急性症状,包括呼吸急促,萎靡不振,咳嗽,发烧,导致他们住院。体格检查显示烦躁,缺氧,呼吸急促,心动过速,和低血压。初始检测利用胸部X光或超声心动图,导致基于心包积液和/或骨髓检查的后续诊断。两名患者在最初诊断时接受了化疗,一种是阿糖胞苷和依托泊苷,另一种是阿糖胞苷和克拉屈滨。后处理,他们的骨髓得到了缓解,在2.5年的随访中,他们的心脏功能仍然良好。不幸的是,其余三名患者在诊断后两周内死亡,由于接受替代药物或没有接受化疗。
    这是第一个也是最大的小儿AML合并心脏MS的病例系列,最初表现为心脏填塞。它强调了与这种情况相关的罕见性和高死亡率。降低死亡率的关键因素包括确定临床表现,进行彻底的身体检查,及时进行超声心动图检查,早期和及时启动心包引流,避免心脏毒性化疗药物。
    UNASSIGNED: This study aims to elucidate the clinical features observed in cases of pediatric acute myeloid leukemia (AML) initially presenting with cardiac tamponade and to share treatment experiences.
    UNASSIGNED: Five pediatric patients were initially diagnosed with AML accompanied by cardiac myeloid sarcoma (MS). The diagnosis was established by examining our hospital records and reviewing pertinent literature from 1990 to July 2023, accessible through MEDLINE/PubMed. We comprehensively assessed the clinical characteristics and treatment modalities employed for these patients.
    UNASSIGNED: Five pediatric patients presented with acute symptoms, including shortness of breath, malaise, cough, and fever, leading to their hospitalization. Physical examination revealed irritability, hypoxia, tachypnea, tachycardia, and hypotension. Initial detection utilized chest X-ray or echocardiogram, leading to subsequent diagnoses based on pericardial effusion and/or bone marrow examination. Two patients received chemotherapy at the time of initial diagnosis, one with cytarabine and etoposide, and the other with cytarabine and cladribine. Post-treatment, their bone marrow achieved remission, and over a 2.5-year follow-up, their cardiac function remained favorable. Unfortunately, the remaining three patients succumbed within two weeks after diagnosis, either due to receiving alternative drugs or without undergoing chemotherapy.
    UNASSIGNED: This is the first and largest case series of pediatric AML patients with cardiac MS, manifesting initially with cardiac tamponade. It highlights the rarity and high mortality associated with this condition. The critical factors for reducing mortality include identifying clinical manifestations, conducting thorough physical examinations, performing echocardiography promptly, initiating early and timely pericardial drainage, and avoiding cardiotoxic chemotherapy medications.
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  • 文章类型: Case Reports
    急性淋巴细胞白血病(ALL)是最常见的儿科恶性肿瘤,约占儿童癌症的25%。尽管治疗方案取得了重大进展,ALL仍然是一种复杂的疾病,经常出现各种并发症,包括罕见的代谢紊乱的B型乳酸性酸中毒。该病例报告详细介绍了一名14岁ALL女性在治疗期间出现B型乳酸性酸中毒的临床历程。病人出现间歇性发热,腹痛,黄疸,和肝脾肿大,伴有严重贫血和血小板减少症。初始管理包括支持治疗和化疗开始。尽管采取了积极的干预措施,病人的病情恶化,随着乳酸性酸中毒和呼吸窘迫的加剧,导致对量身定制的管理策略的迫切需要。该报告强调了早期识别和全面管理小儿ALL中B型乳酸性酸中毒的重要性,强调其多因素病因和潜在威胁生命的后果。增强的临床意识和多学科方法对于改善此类复杂病例的结果至关重要。
    Acute lymphoblastic leukemia (ALL) is the most prevalent pediatric malignancy, accounting for approximately 25% of childhood cancers. Despite significant advancements in treatment protocols, ALL remains a complex disease, often presenting with various complications, including the rare metabolic disturbance of type B lactic acidosis. This case report details the clinical journey of a 14-year-old female with ALL who developed type B lactic acidosis during treatment. The patient presented with intermittent fever, abdominal pain, jaundice, and hepatosplenomegaly, accompanied by severe anemia and thrombocytopenia. Initial management included supportive care and chemotherapy initiation. Despite aggressive interventions, the patient\'s condition deteriorated, with escalating lactic acidosis and respiratory distress, leading to a critical need for tailored management strategies. This report underscores the importance of early recognition and comprehensive management of type B lactic acidosis in pediatric ALL, highlighting its multifactorial etiology and potentially life-threatening consequences. Enhanced clinical awareness and a multidisciplinary approach are crucial for improving outcomes in such complex cases.
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  • 文章类型: Case Reports
    Richter转化(RT)代表先前或同时诊断为慢性淋巴细胞白血病(CLL)的个体中侵入性淋巴瘤的发展,其特征是淋巴结肿大。然而,以结外器官受累为首发症状的病例很少见.没有以乳腺病变为首发症状的RT的报道。非特异性和非典型的临床表现是RT的准确诊断和适当治疗的关键挑战。此病例报告描述了一名老年女性患者,该患者以乳腺病变为首发症状。患者入院时左乳房有无痛肿块。检查发现多发性淋巴结病和异常高的白细胞水平。患者经血液学检查确诊为CLL,骨髓形态学评估,和组织活检.钼靶和B超显示左乳实性占位性病变(BI-RADS5类)。最初,患者拒绝乳腺活检,因此接受了伊布替尼治疗,显示出有限的疗效。受累乳房的穿刺活检表明存在弥漫性大B细胞淋巴瘤。根据辅助和病理检查和病史,最终诊断为RT伴乳腺受累.扎努布替尼联合利妥昔单抗,环磷酰胺,阿霉素,长春新碱,和泼尼松治疗提供初始控制;然而,由于患者病情的波动,治疗策略需要调整。患者的当前状态被标记为稳定,显示出部分缓解的总体成就。患者正在接受后续治疗。我们还对RT进行了全面的文献综述,特别强调它的生物学范式,预后影响,现有的治疗方法,以及治疗方式的新兴方向。
    Richter transformation (RT) represents the development of intrusive lymphoma in individuals previously or concurrently diagnosed with chronic lymphocytic leukemia (CLL) and is characterized by lymph node enlargement. However, cases involving extra-nodal organ involvement as the first symptom are rare. There are no reports of RT with breast lesions as the first symptom. Nonspecific and atypical clinical manifestations represent key challenges in the accurate diagnosis and appropriate treatment of RT. This case report describes an elderly female patient who presented with breast lesions as the first RT symptom. The patient was admitted with a painless mass in the left breast. Examination revealed multiple lymphadenopathies and abnormally high white blood cell levels. The patient was diagnosed with CLL after hematological tests, assessments of bone marrow morphology, and tissue biopsy. Mammography and B-ultrasonography showed solid space-occupying lesions (BI-RADS category 5) in the left breast. Initially, the patient declined a breast biopsy and was therefore prescribed ibrupotinib treatment, which showed limited efficacy. A needle biopsy of the affected breast indicated the presence of diffuse large B-cell lymphoma. Based on auxiliary and pathological examinations and medical history, the final diagnosis was RT with breast involvement. Zanubrutinib with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone treatment provided initial control; however, the treatment strategy required adjustment because of the patient\'s fluctuating condition. The current status of the patient is marked as stable, showing an overall achievement of partial alleviation. The patient is in the process of receiving follow-up treatment. We also performed a comprehensive literature review on RT, with particular emphasis on its biological paradigm, prognosis implications, existing therapeutic approaches, and emerging directions in treatment modalities.
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  • 文章类型: Journal Article
    先前的报道表明,使用Bruton酪氨酸激酶抑制剂(BTKi)治疗慢性淋巴恶性肿瘤,例如慢性淋巴细胞白血病(CLL),可能会增加侵袭性真菌感染(IFIs)的频率。但是缺乏精确的估计。我们的目标是描述CLL患者中IFIs的患病率,BTKi现在是一线推荐的治疗方法。
    我们查询了TriNetX,全球研究网络数据库,使用国际疾病分类来识别患有CLL的成年患者,第十次修订代码(C91.1)和实验室结果。我们进行了病例对照倾向评分匹配分析,以通过BTKi使用确定IFIs事件。我们调整了年龄,性别,种族,和与IFIs风险增加相关的临床风险因素。
    在5358名符合CLL的患者中,我们发现,使用BTKi的患者在5年时发生IFIs的发生率为4.6%,而未使用BTKi的患者中发生IFIs的发生率为3.5%.在此期间,大约有1%的CLL患者在服用BTKi时发生了FI。我们调整后的FI事件分析发现吉罗韦西肺孢子虫肺炎(PJP)的发生率升高(0.5%vs0.3%,P=0.02)和侵袭性念珠菌病(3.5%vs2.7%,P=.012)使用BTKi。对于侵袭性念珠菌病和PJP,服用BTKi的患者需要伤害的人数分别为120和358,分别。
    我们发现使用BTKi的PJP和侵袭性念珠菌病的调整率升高。利率是,然而,低与高数字需要伤害。需要对其他具有特定BTKis的IFIs进行分层的其他研究,以识别有风险的患者并进行预防,具有成本效益的干预措施。
    UNASSIGNED: Prior reports have suggested a possible increase in the frequency of invasive fungal infections (IFIs) with use of a Bruton tyrosine kinase inhibitor (BTKi) for treatment of chronic lymphoid malignancies such as chronic lymphocytic leukemia (CLL), but precise estimates are lacking. We aim to characterize the prevalence of IFIs among patients with CLL, for whom a BTKi is now the first-line recommended therapy.
    UNASSIGNED: We queried TriNetX, a global research network database, to identify adult patients with CLL using the International Classification of Diseases, Tenth Revision code (C91.1) and laboratory results. We performed a case-control propensity score-matched analysis to determine IFIs events by BTKi use. We adjusted for age, sex, ethnicity, and clinical risk factors associated with an increased risk of IFIs.
    UNASSIGNED: Among 5358 matched patients with CLL, we found an incidence of 4.6% of IFIs in patients on a BTKi versus 3.5% among patients not on a BTKi at 5 years. Approximately 1% of patients with CLL developed an IFI while on a BTKi within this period. Our adjusted IFI event analysis found an elevated rate of Pneumocystis jirovecii pneumonia (PJP) (0.5% vs 0.3%, P = .02) and invasive candidiasis (3.5% vs 2.7%, P = .012) with the use of a BTKi. The number needed to harm for patients taking a BTKi was 120 and 358 for invasive candidiasis and PJP, respectively.
    UNASSIGNED: We found an adjusted elevated rate of PJP and invasive candidiasis with BTKi use. The rates are, however, low with a high number needed to harm. Additional studies stratifying other IFIs with specific BTKis are required to identify at-risk patients and preventive, cost-effective interventions.
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    文章类型: Case Reports
    自20XX年10月以来,一名80岁的妇女出现了轻微的发烧和食欲不振。同年11月,病人到我们医院就诊。外周血测试显示存在非典型淋巴细胞,sIL-2R显着增加。骨髓抽吸样品的测试显示CD19,CD20,CD23和λ阳性的小淋巴细胞浸润。因此,诊断为小淋巴细胞淋巴瘤(SLL).在19/20中观察到包括-X和del(13q)的复杂核型。此外,观察到肿大的脾脏和腹膜后肿瘤。作为3个疗程的氟达拉滨加利妥昔单抗治疗的结果,在外周血中不再观察到非典型淋巴细胞,并且肿大的脾脏大小减小。然而,腹膜后肿瘤无法减少。因此,我们于20XX+1年2月对同一区域进行了穿刺活检,并诊断为弥漫性大B细胞淋巴瘤(DLBCL).因为在骨髓中观察到大量CD23阴性淋巴细胞浸润,提示慢性淋巴细胞白血病(CLL)已转化为DLBCL。经过4个疗程的CHOP治疗,腹膜后肿瘤减少。在-X是微克隆的情况下,突变通常与年龄有关。然而,在晚期发生的情况下,就像这个病人身上发生的那样,与造血系统肿瘤的相关性是有争议的。此外,据报道,具有-X的CLL病例与de(l13q)有关。我们的结果强烈表明,带有del(13q)的-X可能是CLL/SLL中的克隆扩增。
    An 80-year-old woman had developed a slight fever and loss of appetite since October 20XX. In November of the same year, the patient visited our hospital. Peripheral blood tests revealed the presence of atypical lymphocytes and a significant increase in sIL-2R. Tests of bone marrow aspiration samples showed the infiltration of small lymphocytes positive for CD19, CD20, CD23, and lambda. Therefore, a diagnosis of small lymphocytic lymphoma(SLL)was made. A complex karyotype including -X and del(13q)was observed in 19/20. Additionally, an enlarged spleen and retroperitoneal tumors were observed. As a result of 3 courses of fludarabine plus rituximab therapy, atypical lymphocytes were no longer observed in the peripheral blood and the enlarged spleen decreased in size. However, the retroperitoneal tumors could not be reduced. Consequently, a needle biopsy from the same area was performed in February 20XX+1, and a diagnosis of diffuse large B-cell lymphoma(DLBCL)was made. Because massive infiltration of CD23-negative lymphocytes was observed in the bone marrow, it was suggested that chronic lymphocytic leukemia(CLL)had transformed into DLBCL. Following 4 courses of CHOP therapy, the retroperitoneal tumors were reduced. In cases where -X is a microclone, the mutation is often age-related. However, in cases of advanced chronogenesis, as occurred in this patient, a correlation with hematopoietic tumors is arguable. Moreover, cases of CLL with -X have been reported to be related to de(l 13q). Our results strongly suggest that -X with del(13q)may be a clonal expansion in CLL/SLL.
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  • 文章类型: Case Reports
    慢性粒细胞白血病(CML)胸腔积液的发展尚不清楚,文献中也很少记录。髓外受累(EMI),发生在大约10%的CML病例中,通常影响淋巴结和脾脏。在CML中很少报道白血病细胞广泛浸润到胸膜中的情况。这里,我们报告了一例41岁的男性患者,在难治性CML的爆发危象期(BC)期间出现显著的双侧胸腔积液伴白血病浸润.胸膜液检查显示具有成髓细胞形态特征的细胞。虽然非常罕见,在CML患者中,应将胸膜白血病浸润视为胸腔积液的原因,尤其是在BC阶段。
    The development of pleural effusion in chronic myeloid leukemia (CML) is not well-understood and rarely documented in literature. Extramedullary involvement (EMI), which occurs in about 10% of CML cases, typically affects lymph nodes and the spleen. Instances of extensive infiltration of leukemic cells into the pleura are infrequently reported in CML. Here, we report a case of 41-year-old man experiencing significant bilateral pleural effusion with leukemic infiltration during the blast crisis (BC) phase of refractory CML. Examination of the pleural fluid revealed cells with morphological characteristics of myeloblasts. Although very rare, pleural leukemic infiltration should be considered as a cause of pleural effusion in patients with CML, especially in the BC phase.
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  • 文章类型: Case Reports
    参与综合征(ES)是造血干细胞移植(HSCT)的早期并发症,其特征是发热和其他临床表现,包括皮疹,腹泻,肺浸润,体重增加,神经症状。HSCT后的类固醇抗性ES显着影响移植的功效,甚至可能导致患者死亡。由于ES基本上代表了由移植的供体细胞诱导的细胞因子风暴,干扰素-γ(IFN-γ)发挥了核心作用,我们假设emaalumab(一种抗IFN-γ单克隆抗体)可能是治疗类固醇耐药ES的有效方法.这里,我们提供一例14岁女性患者的病例报告,该患者因急性髓系白血病复发而接受了第二例单倍体相合HSCT.移植后9天,患者出现发热,对抗菌药物(头孢他啶/阿维巴坦)反应不佳.几天后,病人出现了新发皮疹,体重增加,肝功能受损,导致ES的诊断。最初的免疫抑制(他克莫司和霉酚酸酯)治疗未能控制疾病。移植后第16天,患者接受了两次50mgemaalumab的输注。在emapalumab开始治疗后,患者发热恢复正常,ES得到有效控制。该病例报告表明,emapalumab对类固醇耐药性ES具有可能的疗效,并为治疗这种临床并发症提供了一种新的治疗策略。
    Engraftment syndrome (ES) is an early complication of hematopoietic stem cell transplantation (HSCT) characterized by fever and additional clinical manifestations including rash, diarrhea, lung infiltrates, weight gain, and neurological symptoms. Steroid-resistant ES following HSCT significantly affects the efficacy of transplantation and may even result in patient mortality. As ES essentially represents a cytokine storm induced by engrafted donor cells with interferon-gamma (IFN-γ) playing a central role, we hypothesized that emapalumab (an anti-IFN-γ monoclonal antibody) may be an effective approach to treat steroid-resistant ES. Here, we present a case report of a 14-year-old female patient who received a second haploidentical HSCT due to a relapse of acute myeloid leukemia. Nine days after the transplantation, the patient developed a fever and exhibited a poor response to antimicrobials (ceftazidime/avibactam). A few days later, the patient presented with a new-onset rash, weight gain, and impaired liver function, leading to a diagnosis of ES. Initial immunosuppressive (tacrolimus and mycophenolate mofetil) treatment failed to control the disease. On day 16 post-transplantation, the patient received two infusions of 50 mg of emapalumab. Following the initiation of emapalumab treatment, the patient\'s fever returned to normal and ES was effectively controlled. This case report demonstrated that emapalumab had a possible efficacy for steroid-resistant ES and provided a novel therapeutic strategy to treat this clinical complication.
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  • 文章类型: Case Reports
    急性淋巴细胞白血病(ALL)是一种涉及早期分化淋巴样细胞侵入骨髓的恶性肿瘤,血,和髓外部位。一线治疗跨越2-3年与诱导,合并,强化,长期维护阶段。复发/难治性(R/R)ALL通常具有不良预后,目前没有治疗这种疾病的标准。这里,我们介绍了一例R/RALL对脂质体米托蒽醌多药化疗反应有效的病例,导致35天的治疗后快速完全反应。随后,患者成功接受allo-HSCT治疗.在5个月的随访中,病人还活着,没有白血病。此外,脂质体米托蒽醌治疗或allo-HSCT住院期间未发生严重不良事件.鉴于在我们的案例中观察到的令人鼓舞的疗效和可控制的不良事件,在R/RALL患者中,以米托蒽醌为基础的脂质体多药化疗作为allo-HSCT的桥梁应进一步探索.
    Acute lymphoblastic leukemia (ALL) represents a malignancy involving early-stage differentiated lymphoid cells that invade the bone marrow, blood, and extramedullary sites. First-line treatment spans 2-3 years with induction, consolidation, intensification, and long-term maintenance phases. Relapsed/refractory (R/R) ALL typically carries an adverse prognosis, and there is currently no standard of care for this disease. Here, we present a case of R/R ALL that responded effectively to liposomal mitoxantrone-based multidrug chemotherapy, resulting in a rapid complete response after 35 days of therapy. Subsequently, the patient was successfully treated with allo-HSCT. At 5 months follow-up, the patient was alive and leukemia-free. Additionally, no severe adverse events were recorded during liposomal mitoxantrone treatment or hospitalization for allo-HSCT. Given the encouraging efficacy and the manageable adverse events observed in our case, liposomal mitoxantrone-based multidrug chemotherapy should be further explored as a bridge to allo-HSCT in patients with R/R ALL.
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  • 文章类型: Case Reports
    伊马替尼和尼罗替尼是由bcr-abl融合蛋白产生的酪氨酸激酶(TKIs)的抑制剂,c-Kit,和血小板衍生的生长因子受体。TKI的皮肤不良反应(AE)是最常见的非血液学后遗症。在我们的案例中,共同的分子靶标增加了交叉不耐受的可能性,在这两种药物中都出现类似的AE,可以出现。我们在此报告一例罕见的伊马替尼和尼罗替尼在慢性粒细胞白血病中皮肤AE交叉不耐受的病例报告。
    Imatinib and nilotinib are inhibitors of tyrosine kinases (TKIs) generated from the bcr-abl fusion protein, c-Kit, and platelet-derived growth factor receptors. Cutaneous adverse effects (AEs) of TKI are the most frequent non-hematological sequelae. In our case, the common molecular target raises the possibility that cross-intolerance, in which similar AEs occur with both agents, can arise. We hereby report a rare case report on cross-intolerance of cutaneous AEs of imatinib and nilotinib in chronic myeloid leukemia.
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