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Intermittent Transfusions for Treatment of Thalassemia in the State of Georgia, 2007-2016 2007-2016年格鲁吉亚州治疗地中海贫血的间歇性输血
Pub Date : 2021-04-10 DOI: 10.26420/ANNHEMATOLONCOL.2021.1340
Rollins Mr, J. Boudreaux, J. Eckman, J. Branscomb, M. Zhou, A. Snyder
Background: Individuals with Non-Transfusion Dependent Thalassemia (NTDT) may require infrequent transfusions. Knowing transfusion history, while important, can be challenging in this subgroup. Study Design: Hospital discharge data in Georgia (2007-2016) was reviewed. Thalassemia patients were defined as ≥3 encounters with a thalassemia diagnosis code. Transfusion was defined by the presence of a diagnosis, CPT, revenue, or HCPCS code for red cell transfusion. Results: There were 428 patients identified; 57 received multi-site transfusions. Conclusion: Georgia hospitals provide intermittent transfusions to low volumes of probable NTDT patients. Patient and provider education may help assure adherence to best practices, avoiding serious transfusion complications.
背景:非输血依赖型地中海贫血(NTDT)患者可能不需要频繁输血。了解输血史虽然很重要,但在这个亚组中可能具有挑战性。研究设计:回顾了格鲁吉亚2007-2016年的出院数据。地中海贫血患者定义为遇到地中海贫血诊断代码≥3次。输血的定义是红细胞输血的诊断、CPT、收入或HCPCS代码。结果:共发现428例患者;57例接受多部位输血。结论:乔治亚州的医院为少量可能的NTDT患者提供间歇性输血。对患者和提供者进行教育有助于确保遵守最佳做法,避免严重的输血并发症。
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引用次数: 0
TdT Negativity and ETP Phenotype in Young Patients with Acute T- Lymphoid Leukemia: A Case Report 青年急性T淋巴细胞白血病患者的TdT阴性和ETP表型:一例报告
Pub Date : 2021-04-05 DOI: 10.26420/ANNHEMATOLONCOL.2021.1339
Cetin D, Midik Mm, Karadag Fk, Ozsan N, Pariltay E, Akin H, Saydam G
TdT is generally positive in patients with T-Acute Lymphoblastic Leukemia/ Lymphoma (T-ALL/LBL) and can often become negative after chemotherapy. TdT negativity at the time of diagnosis is not a common condition and is evaluated in favor of a poor prognosis. Due to its infrequent occurrence, there are not enough clinical studies, and there are often publications on the basis of case and case series. While its incidence increases in young people and children, the possibility of accompanying Early T Precursor (ETP) phenotype also increases. The presence of the ETP phenotype has negative repercussions on prognosis. Here, we will describe the diagnosis and treatment process of a young adult patient with TdT negative T-ALL accompanied by the ETP phenotype.
TdT在T型急性淋巴细胞白血病/淋巴瘤(T-ALL/LBL)患者中通常呈阳性,并且在化疗后通常会变为阴性。诊断时TdT阴性不是一种常见的情况,并且被评估为预后不良。由于其罕见,没有足够的临床研究,经常有基于病例和病例系列的出版物。虽然其在年轻人和儿童中的发病率增加,但伴随早期T前体(ETP)表型的可能性也增加。ETP表型的存在对预后有负面影响。在这里,我们将描述一名患有TdT阴性T-ALL并伴有ETP表型的年轻成年患者的诊断和治疗过程。
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引用次数: 0
Hemophagocytic Lymphohistiocytosis after Initiation of Combined Immunotherapy for Metastatic Melanoma 开始联合免疫治疗转移性黑色素瘤后的噬血细胞淋巴组织细胞增多症
Pub Date : 2021-03-29 DOI: 10.26420/ANNHEMATOLONCOL.2021.1337
T. Gambichler, Rached Na, N. Nowack, B. Behle, L. Susok
Hemophagocytic Lymphohistiocytosis (HLH), which is a severe, potentially fatal condition characterized by T lymphocyte overactivation, is predominantly caused by infections, hematological malignancies, and autoimmune conditions. HLH due to therapy with Immune Checkpoint Inhibitors (ICI) has rarely been reported. We describe a 60-year-old male with metastatic melanoma who developed HLH after the initiation of nivolumab plus ipilimumab treatment. Prompt diagnosis and high-dose mono-prednisolone therapy resulted in rapid resolution of his subjective symptoms and laboratory findings. Apart from this case presentation we provide a brief overview on clinical characteristics of previously observed ICI-induced HLH cases. Given the increasing use of ICI in a variety of cancers, the frequency of HLH will very likely raise. HLH morbidity and mortality are often the result of delayed diagnosis and inappropriate treatment. Hence, HLH must be considered in ICI-treated cancer patients who present with symptoms such as fever, cytopenias and hyperferritinemia.
吞噬血淋巴细胞增多症(HLH)是一种以T淋巴细胞过度活化为特征的严重、潜在的致命疾病,主要由感染、血液系统恶性肿瘤和自身免疫性疾病引起。用免疫检查点抑制剂(ICI)治疗HLH的报道很少。我们描述了一名患有转移性黑色素瘤的60岁男性,他在开始nivolumab加ipilimumab治疗后出现HLH。及时的诊断和高剂量的泼尼松治疗使他的主观症状和实验室检查结果迅速得到解决。除此病例介绍外,我们还简要概述了先前观察到的ICI诱导的HLH病例的临床特征。鉴于ICI在各种癌症中的使用越来越多,HLH的频率很可能会升高。HLH的发病率和死亡率通常是诊断延迟和治疗不当的结果。因此,在ICI治疗的癌症患者中,出现发烧、血细胞减少和高铁蛋白血症等症状时,必须考虑HLH。
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引用次数: 1
Massive Bone Marrow Carcinomatosis Presenting with Leukoerythroblastic Blood Reaction 以白细胞-红细胞血液反应为表现的大规模骨髓癌
Pub Date : 2021-03-25 DOI: 10.26420/ANNHEMATOLONCOL.2021.1336
Ana Garzó, J. VerduBelmar, E. Campos, De Paz Fj, F. Tarín
We present a case of an extensive bone marrow infiltrate of poorly differentiated carcinoma, in a 19-year-old male with suspected progression of sinusal undifferentiated carcinoma, after several lines of treatment.
我们报告了一例低分化癌的广泛骨髓浸润病例,该病例发生在一名19岁男性身上,在经过几系列治疗后,疑似进展为窦性未分化癌。
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引用次数: 0
EBV-Associated Post-Transplant Lymphoproliferative Disease (PTLD) in Allogeneic Transplantation 同种异体移植中ebv相关的移植后淋巴增生性疾病(PTLD)
Pub Date : 2021-03-16 DOI: 10.26420/ANNHEMATOLONCOL.2021.1335
Cutini I, Peruzzi B, Caporale R, Nozzoli C, Gozzini A, Innocenti C, Boncompagni R, Fani A, Saccardi R
Post-Transplant Lymphoproliferative Disease (PTLD) following both Solid Organ Transplantation (SOT) and Hematopoietic Stem Cell Transplantation (HSCT) is a rare life-threatening complication. The majority of PLTDs are associated to Epstein Bar Virus (EBV) [1] reactivation, usually in the early phase [2] after transplant, when the patient is severely immunocompromised and is unable to control virus replication [3]. Despite the mortality of EBV-associated PTLD has been reduced over the years, the different histological patterns of its presentation, ranging from indolent to high grade B cell lymphoma, still play a role in the outcome. Herein, we report the case of a 60-years-old man diagnosed with acute myeloid leukemia who underwent allogeneic transplantation and developed a fatal Hemophagocytic Histiocytosis (HLH) secondary to an aggressive EBV-associated PTLD, not responding to a rituximab-based treatment.
实体器官移植(SOT)和造血干细胞移植(HSCT)后的移植后淋巴增生性疾病(PTLD)是一种罕见的危及生命的并发症。大多数pltd与eb病毒(EBV)[1]再激活有关,通常发生在移植后的早期阶段,此时患者严重免疫功能低下,无法控制病毒复制[3]。尽管ebv相关PTLD的死亡率多年来一直在降低,但其表现的不同组织学模式,从惰性到高级别B细胞淋巴瘤,仍然在结果中发挥作用。在此,我们报告了一例60岁的男性诊断为急性髓性白血病,他接受了同种异体移植,并发了致命性的噬血细胞组织细胞增生症(HLH),继发于侵袭性ebv相关的PTLD,利妥昔单抗治疗无效。
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引用次数: 0
IgG4-Related Disease and Successful Treatment with Rituximab in a Three-Year-Old Boy IgG4相关疾病与利妥昔单抗在一名三岁男孩中的成功治疗
Pub Date : 2021-03-16 DOI: 10.26420/ANNHEMATOLONCOL.2021.1334
J. Marissen, Julia Pagel, A. Steinmetz, C. Härtel, M. Lauten
IgG4-Related Disease (IgG4-RD) is a rare inflammatory disease, which may affect all organs and lead to severe fibrosis and organ damage. Mainly described in Japanese or Asian adults, case reports on children-variously affected by the disease-are increasing worldwide. While glucocorticoids are broadly used as first line treatment, several cases require second line options due to relapse or resistance to first line treatment. We report a boy who was diagnosed with IgG4-related disease at the age of three years. He presented an IgG4-associated tumorous lesion in the upper lobe of the left lung, pleural and pericardial effusion and enlarged abdominal lymph nodes. Therapy with surgery and glucocorticoids followed by rituximab treatment led to continuous complete remission 27 months after diagnosis of IgG4-RD.
igg4相关疾病(IgG4-RD)是一种罕见的炎症性疾病,可影响所有器官,导致严重的纤维化和器官损害。主要发生在日本或亚洲的成年人中,世界范围内受各种疾病影响的儿童病例报告正在增加。虽然糖皮质激素广泛用作一线治疗,但由于复发或对一线治疗产生耐药性,一些病例需要二线治疗。我们报告了一个三岁时被诊断患有igg4相关疾病的男孩。他在左肺上叶出现igg4相关的肿瘤病变,胸膜和心包积液,腹部淋巴结肿大。在诊断出IgG4-RD后27个月,手术和糖皮质激素治疗以及利妥昔单抗治疗导致持续完全缓解。
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引用次数: 0
Unusual Manifestations of Paraprotein-Associated Hematological Diseases: Report of 2 Cases and Literature Review 副蛋白相关血液病的异常表现2例报告并文献复习
Pub Date : 2021-03-11 DOI: 10.26420/ANNHEMATOLONCOL.2021.1333
Karakatsanis S, Papadatos Ss, Polyzos D, Gravani A, Syrigos N, Tsakania A, Kanellis G, M. L, Papanikolaou A
With this review we firstly describe uncommon manifestations of paraprotein-associated hematological diseases in 2 patients presenting with hemoptysis. The differential diagnosis in both cases included qualitative and quantitative platelet abnormalities, infectious diseases, malignancies, vasculitis, collagen diseases and blood disorders. Among other interventions undertaken, bone marrow biopsy provided evidence of a primary hematological disease while serum protein electrophoresis and immunofixation indicated the presence of a paraprotein. As a “take-away” lesson from the literature review performed on the basis of these cases the bleeding diathesis that is occasionally associated with paraproteinemia should be pointed out, irrespectively of the primary hematological condition. Thus, while investigating a patient with bleeding diathesis, at least a serum protein electrophoresis should be among the requested laboratory tests. Moreover, clinical vigilance is required in order to recognize other unusual manifestations of not so unusual hematological abnormalities.
在这篇综述中,我们首次描述了2例以咳血为特征的患者的副蛋白相关血液系统疾病的罕见表现。这两种病例的鉴别诊断包括定性和定量血小板异常、传染病、恶性肿瘤、血管炎、胶原疾病和血液疾病。在进行的其他干预措施中,骨髓活检提供了原发性血液病的证据,而血清蛋白电泳和免疫固定表明存在副蛋白。作为从基于这些病例进行的文献综述中“吸取”的教训,应指出偶尔与副蛋白血症相关的出血素质,而不考虑原发性血液病。因此,在调查具有出血素质的患者时,至少应在要求的实验室测试中进行血清蛋白电泳。此外,临床上需要保持警惕,以识别其他不常见的血液学异常的异常表现。
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引用次数: 0
Molecular Screening of Hemoglobin D Variant in Anemia Patients of Eastern UP Population, India 印度东部UP人群贫血患者血红蛋白D变体的分子筛查
Pub Date : 2021-03-05 DOI: 10.26420/ANNHEMATOLONCOL.2021.1330
Rai, U. Yadav, P. Kumar, Mishra Op
Hemoglobinopathy is the commonest inherited monogenic disorders, which are highly prevalent in India. The three most predominant hemoglobinopathies are HbS, HbE, the aim of the present study is to determine the frequency of HbD in Eastern UP population. After taking written consent, blood samples was collected from 350 individuals and genomic DNA was extracted from a the collected blood samples. PCR-RFP method was used to analyze the mutation. Out of 350 samples analyzed, one individual was heterozygous (HbD/N) and two individuals were homozygous (HbD/D) for Hb D mutation. In conclusion, the overall βD allele frequency in Eastern Uttar Pradesh was observed as 0.71%.
血红蛋白病是最常见的遗传性单基因疾病,在印度非常普遍。三种最主要的血红蛋白病是HbS和HbE,本研究的目的是确定东部UP人群中HbD的频率。在取得书面同意后,从350人中采集血样,并从采集的血样中提取基因组DNA。采用PCR-RFP方法进行突变分析。在分析的350个样本中,一个个体是Hb D突变的杂合子(HbD/N),两个个体是纯合子(HbD)。总之,北方邦东部的βD等位基因总频率为0.71%。
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引用次数: 0
Synchronous Occurrence of Hodgkin’s Lymphoma and Lung Cancer 霍奇金淋巴瘤与肺癌同时发生
Pub Date : 2021-03-05 DOI: 10.26420/ANNHEMATOLONCOL.2021.1332
R. Erdem, F. Aykaş, M. Göçer, O. In, Aslan, Mustafa Celik, G. Cetin, E. Kurtoğlu
Hodgkin lymphoma is an uncommon neoplasm that characterized young age of onset, Hodgkin and Reed-Sternberg (HRS) cells derived from B-lymphocytes and a high cure rate, even when the patient presents with advanced metastatic spread. Lung cancer is the most common cancer worldwide and is still responsible for the most cancer deaths. We present an extremely rare case of coexisting Hodgkin lymphoma and lung cancer in a 67-year-old male patient. He initially presented with chest pain. Pet/ct revealed mass in the right lung and lymph nodes in the neck. Biopsy from the premaxillary lymph node was compatible with classical Hodgkin lymphoma. In terms of second primary malignancy, a biopsy was also performed from the mass in the right lung. Pathology showed a pulmonary adenocarcinoma and a right upper lobectomy was then performed. This patient was treated with gemcitabine plus docetaxel for lung cancer. At the end of treatment pet/ct was complete response including lymph nodes in the neck. Therefore, we did not give any treatment for Hodgkin lymphoma. The patient is still being followed up in remission.
霍奇金淋巴瘤是一种罕见的肿瘤,其特点是发病年龄小,霍奇金和里德-斯特恩伯格(HRS)细胞来源于b淋巴细胞,即使患者出现晚期转移扩散,治愈率也很高。肺癌是世界上最常见的癌症,也是导致最多癌症死亡的原因。我们报告一例罕见的霍奇金淋巴瘤和肺癌并存的病例,患者为67岁男性。他最初表现为胸痛。Pet/ct示右肺肿块及颈部淋巴结。上颌前淋巴结活检符合经典霍奇金淋巴瘤。对于第二原发恶性肿瘤,我们也对右肺肿块进行了活检。病理显示为肺腺癌,随后行右上叶切除术。该患者接受吉西他滨加多西紫杉醇治疗肺癌。治疗结束时pet/ct显示完全缓解,包括颈部淋巴结。因此,我们没有对霍奇金淋巴瘤进行任何治疗。病人仍在接受病情缓解的随访。
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引用次数: 1
School Personnel’s Self-Esteem, Sense of Self-Efficacy and Interventions on Weight-Related Bullying According to Their Weight Perception and Dieting Behaviors 学校人员的自尊、自我效能感及体重感知和节食行为对体重相关欺凌的干预
Pub Date : 2021-02-18 DOI: 10.26420/annhematoloncol.2021.1328
A. A., G. C, Maiano C, Robert Mazaye C, LeBlanc L
To increase academic performance in children, elementary school personnel are encouraged to focus on socio-emotional learning. Better classroom management and safer environments, exempt of bullying and particularly of weight-related bullying, appear like ways of fostering socio-emotional learning in children. However, some school personnel’s characteristics could impact their ability to act on these dimensions. This research is interested in how weightrelated intervention behaviors, self-esteem and sense of self-efficacy vary according to school personnel’s dieting behaviors and weight perception are related to their self-esteem, sense of self-efficacy, and intervention behaviors on weight-related bullying. A total of 164 Canadian participants filled in questionnaires focusing on bullying, self-esteem, and sense of self-efficacy. Results show that most school personnel felt competent to manage their group of students and to intervene on weight-related bullying. Those who were on a diet and who perceived their weight as higher seem significantly more involved in promoting motivation for school and learning engagement in their students as well as more likely to intervene with the bully when encountering weight-related bullying situations. For their part, participants of normal weight who were on a diet had a significantly lower self-esteem than those dieting and having a perception of overweight. These results are encouraging because they suggest that elementary school personnel feel competent with regards to the socioemotional learning of their students and is actively involved in providing them a safe learning environment.
为了提高孩子们的学习成绩,小学工作人员被鼓励关注社会情感学习。更好的课堂管理和更安全的环境,避免欺凌,特别是与体重有关的欺凌,似乎是促进儿童社会情感学习的方法。然而,一些学校人员的特点可能会影响他们在这些方面的行动能力。本研究关注体重相关干预行为、自尊和自我效能感如何随学校人员节食行为而变化,体重感知与自尊、自我效能感和体重相关欺凌干预行为相关。共有164名加拿大参与者填写了关于欺凌、自尊和自我效能感的调查问卷。结果显示,大多数学校工作人员认为有能力管理他们的学生群体并干预与体重有关的欺凌行为。那些正在节食并认为自己体重较高的人似乎更能促进学生的学习动机和学习参与,并且在遇到与体重有关的欺凌情况时更有可能干预欺凌者。对于正常体重的参与者来说,他们的自尊心明显低于那些正在节食并有超重感觉的人。这些结果是令人鼓舞的,因为它们表明小学工作人员在学生的社会情感学习方面感到有能力,并积极参与为他们提供安全的学习环境。
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引用次数: 0
期刊
Annals of hematology & oncology
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