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Complete Remission with Inotuzumab Ozogamicin as Fourth-Line Salvage Therapy in a Child with Relapsed/Refractory Acute Lymphoblastic Leukemia. 复发/难治性急性淋巴细胞白血病患儿接受因妥珠单抗奥佐加米星四线挽救疗法后病情完全缓解
IF 1.1 Q4 HEMATOLOGY Pub Date : 2024-09-27 DOI: 10.3390/hematolrep16040056
Athanasios Tragiannidis, Vassiliki Antari, Eleni Tsotridou, Theodoros Sidiropoulos, Aikaterini Kaisari, Maria Palabougiouki, Timoleon-Achilleas Vyzantiadis, Emmanuel Hatzipantelis, Assimina Galli-Tsinopoulou, Evgenios Goussetis

Background: Despite the progress achieved regarding survival rates in childhood acute lymphoblastic leukemia (ALL), relapsed or refractory disease still poses a therapeutic challenge. Inotuzumab ozogamicin is a CD22-directed monoclonal antibody conjugated to calicheamicin, which has been approved by the Food and Drug Administration for adults and pediatric patients 1 year and older with relapsed or refractory CD22-positive B-cell precursor acute lymphoblastic leukemia. Case presentation: Herein, we present the case of a 23-month-old girl with high-risk B-ALL who experienced very early isolated medullary relapse; following the failure of conventional chemotherapy according to the ALL-IC REL 2016 protocol, she went on to receive the bispecific T-cell engager (BiTE) blinatumomab and subsequently, due to refractory disease, the combination of fludarabine, cytarabine, and the proteasome inhibitor bortezomib without achieving remission. Given the high CD22 expression by the lymphoblasts, off-label use of inotuzumab ozogamicin (InO) was chosen and administrated in a 28-day cycle as a salvage treatment. The minimal residual disease (MRD) was 0.08% on day 28, and InO was continued, thus achieving MRD negativity; the patient successfully underwent an allogeneic stem cell transplantation from a matched family donor. Conclusions: Our case highlights the efficacy and safety of InO as a salvage treatment in the setting of relapsed B-ALL refractory not only to conventional chemotherapy but also to novel treatments, such as blinatumomab and bortezomib.

背景:尽管在儿童急性淋巴细胞白血病(ALL)的存活率方面取得了进展,但复发或难治性疾病仍然是一个治疗难题。Inotuzumab ozogamicin是一种CD22定向单克隆抗体,与卡利昔明共轭,已被美国食品药品管理局批准用于治疗1岁及以上复发或难治性CD22阳性B细胞前体急性淋巴细胞白血病成人和儿童患者。病例介绍:在此,我们介绍了一例 23 个月大的高危 B-ALL 女孩的病例,她经历了非常早期的孤立髓质复发;根据 ALL-IC REL 2016 方案,在常规化疗失败后,她继续接受了双特异性 T 细胞诱导剂(BiTE)blinatumomab,随后,由于疾病难治,她又接受了氟达拉滨、阿糖胞苷和蛋白酶体抑制剂硼替佐米的联合治疗,但未取得缓解。鉴于淋巴母细胞的 CD22 高表达,患者选择了标签外使用伊妥珠单抗-奥佐加米星(InO),以 28 天为一个周期进行挽救性治疗。第28天的最小残留病(MRD)为0.08%,继续使用InO,从而实现了MRD阴性;患者成功接受了匹配的家族捐献者的异基因干细胞移植。结论:我们的病例凸显了InO作为复发B-ALL抢救治疗的有效性和安全性,它不仅对常规化疗难治,而且对新型治疗(如blinatumomab和硼替佐米)也难治。
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引用次数: 0
Padua Prediction Score and Hospital-Acquired Proximal and Isolated Distal Deep Vein Thrombosis in Symptomatic Patients. 帕多瓦预测评分与无症状患者在医院获得的近端和孤立远端深静脉血栓。
IF 1.1 Q4 HEMATOLOGY Pub Date : 2024-09-25 DOI: 10.3390/hematolrep16040055
Michelangelo Sartori, Miriam Fiocca, Mario Soldati, Laura Borgese, Elisabetta Favaretto, Benilde Cosmi

Background: Hospital-acquired deep vein thrombosis (DVT) is an important cause of morbidity and mortality.

Objectives: The purpose of this study was to evaluate the prevalence of proximal lower limb DVT and isolated distal DVT (IDDVT) and their relationship to the Padua Prediction Score (PPS) in acutely ill, hospitalized patients.

Methods: In a single-center cross-sectional study, all inpatients from medical departments with suspected lower-extremity DVT were evaluated with whole-leg ultrasonography during 183 days from 2016 to 2017.

Results: Among the 505 inpatients (age 78.0 ± 13.3, females 59.2%) from medical departments, 204 (40.2%) had PPS ≥ 4, but only 54.4% of them underwent pharmacological thrombo-prophylaxis. Whole-leg ultrasonography detected 47 proximal DVTs (9.3%) and 65 IDDVTs (12.8%). Proximal DVT prevalence was higher in patients with high PPS vs. those with low PPS (12.7% vs. 7.0% p = 0.029, respectively), whereas IDDVT prevalence was similar in patients with high and low PPS (14.7% vs. 11.6% p = 0.311, respectively). The area under the receiver operating curve (AUC) for the PPS was 0.62 ± 0.03 for all DVTs, 0.64 ± 0.04 for proximal DVTs, and 0.58 ± 0.04 for IDDVTs.

Conclusions: In hospitalized patients, IDDVT had similar prevalence regardless of PPS risk stratification. Adherence to thrombo-prophylaxis in patients was still far from optimal.

背景:医院获得性深静脉血栓(DVT)是发病和死亡的重要原因:医院获得性深静脉血栓(DVT)是发病和死亡的重要原因:本研究旨在评估急性住院患者下肢近端深静脉血栓和孤立远端深静脉血栓(IDDVT)的发病率及其与帕多瓦预测评分(PPS)的关系:在一项单中心横断面研究中,在2016年至2017年的183天内,对内科所有疑似下肢深静脉血栓的住院患者进行了全腿超声评估:在 505 名内科住院患者(年龄 78.0 ± 13.3,女性 59.2%)中,204 人(40.2%)的 PPS ≥ 4,但只有 54.4%的患者接受了药物血栓预防治疗。全腿超声波检查发现了 47 例近端深静脉血栓(9.3%)和 65 例 IDDVT(12.8%)。高 PPS 患者的近端深静脉血栓发生率高于低 PPS 患者(分别为 12.7% 对 7.0% p = 0.029),而高 PPS 患者和低 PPS 患者的 IDDVT 发生率相似(分别为 14.7% 对 11.6% p = 0.311)。所有深静脉血栓的PPS接收者操作曲线下面积(AUC)为0.62 ± 0.03,近端深静脉血栓为0.64 ± 0.04,IDDVT为0.58 ± 0.04:结论:在住院患者中,无论PPS风险分层如何,IDDVT的发病率都相似。结论:在住院患者中,无论PPS风险分层如何,IDDVT的发病率都很接近,患者对血栓预防的依从性仍远未达到最佳水平。
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引用次数: 0
How We Treat Hemolytic Anemia Due to Pyruvate Kinase Deficiency. 我们如何治疗丙酮酸激酶缺乏引起的溶血性贫血?
IF 1.1 Q4 HEMATOLOGY Pub Date : 2024-08-31 DOI: 10.3390/hematolrep16030054
Sara Tama-Shekan, Valeria Moreno, Ludovic Saba, Chakra P Chaulagain

Background: Pyruvate kinase (PK) deficiency is an inherited red blood cell (RBC) enzyme disorder that results in non-immune chronic hemolytic anemia. Characteristic symptoms of PK deficiency include anemia, fatigue, splenomegaly, jaundice, gallstones, thrombosis, and transfusional iron overload. Previously, treatments aimed at symptomatic management with RBC transfusions, phototherapy, folic acid supplementation, splenectomy, and iron chelation therapy when iron overload was documented. Mitapivat, a recently approved medication for treatment of PK-deficiency hemolytic anemia, is an oral allosteric activator of wild-type and mutant RBC PK enzymes. In this paper, we describe three cases of PK-deficiency anemia treated with mitapivat and describe modern management of this rare hemolytic disorder.

Methods: A retrospective healthcare database analysis was conducted to extract relevant information. Both quantitative and qualitative methods were integrated to provide a more comprehensive understanding of the cases.

Results: Two patients responded well to treatment with mitapivat, noted by an increase in hemoglobin levels, improvements in hemolytic markers, less frequent or no RBC transfusion requirements, and improvements in fatigue. One patient carrying two non-missense mutations of the PKLR gene did not respond to treatment with mitapivat. As variations in patient-specific factors (including genotype) can lead to different clinical manifestations and responses to treatment, we recommend considering both the phenotype (clinical symptoms and signs) and the genotype of the PKLR gene when making therapeutic decisions about starting a patient on mitapivat.

Conclusions: While mitapivat addresses the previously unmet needs of most patients with PK deficiency as the first and only disease-modifying medication to receive approval for this condition, not all patients with PK deficiency are amenable to treatment with mitapivat.

背景:丙酮酸激酶(PK)缺乏症是一种遗传性红细胞(RBC)酶疾病,会导致非免疫性慢性溶血性贫血。PK缺乏症的特征性症状包括贫血、乏力、脾肿大、黄疸、胆结石、血栓形成和输血铁超载。以前的治疗方法主要是通过输注红细胞、光疗、补充叶酸、脾切除术和铁超载时的铁螯合疗法来对症治疗。米他匹伐是最近批准用于治疗PK缺陷性溶血性贫血的药物,它是一种口服的野生型和突变型红细胞PK酶异位激活剂。本文描述了用米他匹伐治疗PK缺陷性贫血的三个病例,并介绍了这种罕见溶血性疾病的现代治疗方法:方法:对医疗数据库进行回顾性分析,提取相关信息。方法:对回顾性医疗数据库进行分析,提取相关信息,并结合定量和定性方法,以更全面地了解病例:两名患者对米他匹伐治疗反应良好,表现为血红蛋白水平升高、溶血标志物改善、输注红细胞次数减少或无需输注红细胞以及疲劳感改善。一名携带两个 PKLR 基因非缺失突变的患者对米他匹伐治疗没有反应。由于患者特异性因素(包括基因型)的变化会导致不同的临床表现和治疗反应,因此我们建议在决定是否让患者开始使用米他匹伐治疗时,同时考虑表型(临床症状和体征)和PKLR基因的基因型:尽管米他匹伐作为首个也是唯一一个获得批准用于治疗PK缺乏症的改变病情药物,满足了大多数PK缺乏症患者之前未得到满足的需求,但并非所有PK缺乏症患者都适合接受米他匹伐治疗。
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引用次数: 0
Hyperbaric Oxygen Therapy during Pregnancy for Critical Anemia Secondary to Sickle Cell Disease with Post-Transfusion Hyperhemolysis: A Case Report. 妊娠期高压氧疗法治疗镰状细胞病继发的重症贫血和输血后高溶血:病例报告。
IF 1.1 Q4 HEMATOLOGY Pub Date : 2024-08-30 DOI: 10.3390/hematolrep16030053
Shawn Khan, Connor T A Brenna, Jacob Pendergrast, A Kinga Malinowski, Marcus Salvatori, Rita Katznelson, Jordan Tarshis

Background: Sickle cell disease is the most common human monogenetic disease, and its risks are amplified during pregnancy. Methods: This report describes a 35-year-old woman with HgbSS sickle cell disease who developed hyperhemolysis syndrome after undergoing an exchange transfusion during pregnancy. Results: In addition to conventional medical treatment, the patient received prepartum hyperbaric oxygen therapy (HBOT), totaling 17 treatments for the indication of severe anemia. She experienced significant clinical improvement while undergoing HBOT and ultimately delivered a healthy preterm infant by cesarean section. Conclusions: The risks, benefits, and challenges of using HBOT in this unique context are discussed.

背景:镰状细胞病是人类最常见的单基因遗传病,其风险在妊娠期会增大。研究方法本报告描述了一名患有 HgbSS 镰状细胞病的 35 岁女性在妊娠期间接受交换输血后出现高溶血综合征的情况。结果除常规药物治疗外,患者还接受了产前高压氧治疗(HBOT),共治疗 17 次,以缓解严重贫血症状。在接受高压氧治疗期间,她的临床症状得到了明显改善,并最终通过剖腹产产下了一名健康的早产儿。结论:本文讨论了在这种特殊情况下使用 HBOT 的风险、益处和挑战。
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引用次数: 0
IgG-k/IgG-λ Para-Osseous Plasmacytoma Relapsed as Soft-Tissue Plasmacytoma with IgA-k Immunophenotype: A Case Report and Review of the Literature on Related Biochemical Aspects. IgG-k/IgG-λ副骨浆细胞瘤复发为具有 IgA-k 免疫表型的软组织浆细胞瘤:病例报告及相关生化方面的文献综述。
IF 1.1 Q4 HEMATOLOGY Pub Date : 2024-08-29 DOI: 10.3390/hematolrep16030052
Manlio Fazio, Chiara Maria Catena Sorbello, Vittorio Del Fabro, Alessandra Romano, Maria Teresa Cannizzaro, Nunziatina Laura Parrinello, Benedetta Esposito, Sara Frazzetto, Federica Elia, Francesco Di Raimondo, Concetta Conticello

Neoplastic plasma cells (PCs) proliferation at anatomic sites dislocated from the bone marrow (BM) or their contiguous growth from osseous lesions that disrupt the cortical bone is termed extramedullary multiple myeloma (EMD). EMD still remains challenging from a therapeutic and biological perspective. Pathogenesis has not been completely clarified, and it is generally associated with high-risk cytogenetics (HRCAs). In order to emphasize the clinical and biochemical complexity of this disease, we have decided to describe the case of a patient affected by relapsed-refractory (RR) EMD, which presented as para-osseous plasmacytoma with a bi-phenotypical immunoglobulin (Ig) component and lately relapsed as soft-tissue plasmacytoma with a total immunophenotype switch. We have also hypothesized a correlation between Ig patterns and prognosis and suggested the possible inclusion of these biochemical features in the general risk assessment.

髓外多发性骨髓瘤(EMD)是指肿瘤性浆细胞(PCs)在骨髓(BM)脱位的解剖部位增殖,或从破坏骨皮质的骨质病变处毗连生长。从治疗和生物学角度来看,髓外多发性骨髓瘤仍具有挑战性。其发病机制尚未完全明确,通常与高危细胞遗传学(HRCA)有关。为了强调这种疾病在临床和生化方面的复杂性,我们决定描述一例复发-难治性(RR)EMD 患者的病例,该患者表现为具有双表型免疫球蛋白(Ig)成分的骨旁浆细胞瘤,最近复发为具有完全免疫表型转换的软组织浆细胞瘤。我们还假设了 Ig 模式与预后之间的相关性,并建议将这些生化特征纳入一般风险评估。
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引用次数: 0
First-Line Combination with Proteasome Inhibitor-Based Treatment and Zoledronic Acid Is Effective in Reducing Later Fractures in Multiple Myeloma Irrespective of Multiple Myeloma Bone Disease at Diagnosis. 蛋白酶体抑制剂和唑来膦酸一线联合治疗可有效减少多发性骨髓瘤后期骨折,与诊断时的多发性骨髓瘤骨病无关。
IF 1.1 Q4 HEMATOLOGY Pub Date : 2024-08-06 DOI: 10.3390/hematolrep16030051
Veera Eskelinen, Elise Nivakoski, Kirsi Launonen, Anu Partanen, Sakari Kakko, Milla E L Kuusisto

The present study provides real-world evidence on the treatment of multiple myeloma (MM) bone disease with various bisphosphonates combined for different myeloma-specific treatments as no validated data regarding the best combination treatment for bone disease associated with MM are available. We examined retrospectively 345 MM patients treated with autologous stem cell transplantation in Finland during 1996-2020. The median age of the patients was 60 years with a median follow-up time of 50 months (1-339). At diagnosis, 72.1% of the patients had myeloma-associated bone disease and 45.8% had fractures. Most patients (58.8%) received proteasome inhibitor (PI)-containing treatment at first line. MM bone disease was treated in 91.6% of the patients; 49.9% received zoledronic acid (ZA) and 29.9% pamidronate. Inferior overall survival was associated with MM bone disease at diagnosis (p = 0.005) or a fracture at diagnosis (p = 0.003). A later fracture was identified in 29% of the patients, and in those patients without MM bone disease at diagnosis later fractures were less common after ZA treatment (p = 0.049). PI-based treatment plus ZA (p = 0.019) seemed to be the best combination to prevent later fractures, even though the same patient subgroup was more likely to experience relapse (p = 0.018), and also when excluding patients with previous induction therapy without novel agents (p = 0.008). To conclude, this study suggests that the best therapy to prevent later fractures in MM might be PI-based treatment combined with ZA.

由于目前还没有关于治疗多发性骨髓瘤(MM)相关骨病的最佳联合疗法的有效数据,本研究提供了使用各种双膦酸盐联合不同骨髓瘤特异性疗法治疗多发性骨髓瘤(MM)骨病的实际证据。我们回顾性研究了1996-2020年间在芬兰接受自体干细胞移植治疗的345名MM患者。患者的中位年龄为60岁,中位随访时间为50个月(1-339)。确诊时,72.1%的患者患有骨髓瘤相关骨病,45.8%的患者有骨折。大多数患者(58.8%)接受了含蛋白酶体抑制剂(PI)的一线治疗。91.6%的患者接受了骨髓瘤骨病治疗;49.9%接受了唑来膦酸(ZA)治疗,29.9%接受了帕米膦酸盐治疗。总生存率较低与确诊时患有 MM 骨病(p = 0.005)或确诊时骨折(p = 0.003)有关。29%的患者后来发生了骨折,在诊断时没有MM骨病的患者中,ZA治疗后发生骨折的比例较低(p = 0.049)。以 PI 为基础的治疗加 ZA(p = 0.019)似乎是预防后期骨折的最佳组合,尽管同一患者亚群更有可能复发(p = 0.018),而且在排除既往接受过诱导治疗但未使用新型药物的患者时也是如此(p = 0.008)。总之,本研究表明,预防MM日后骨折的最佳疗法可能是基于PI的治疗联合ZA。
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引用次数: 0
Lymphocytic Lymphoma Transforming into Hodgkin Lymphoma in Sub-Saharan Africa: Case Report and Literature Review. 撒哈拉以南非洲的淋巴细胞淋巴瘤转变为霍奇金淋巴瘤:病例报告和文献综述。
IF 1.1 Q4 HEMATOLOGY Pub Date : 2024-08-05 DOI: 10.3390/hematolrep16030050
Sokhna Aïssatou Touré, Dibor Niang, Serigne Mourtalla Gueye, Mohamed Keita, Alioune Badara Diallo, Elimane Seydi Bousso, Fatma Dieng, Blaise Felix Faye, Moussa Seck, Saliou Diop

The Hodgkin variant Richter syndrome (HvRS) is an infrequent complication occurring in 1% of lymphocytic lymphoma/chronic lymphocytic leukemia patients. We report a case of HvRS diagnosed in Sub-Saharan Africa. A 63-year-old patient was consulted for the investigation of an abdominal mass that had been evolving for 5 years prior to admission. His history revealed night sweats, 13% weight loss in 3 months and persistent pruritis. Examination revealed bilateral cervical axillary and inguinal macroadenopathies, painless abdominal distension, pruritic lesions and WHO 2 PS. The blood count showed anemia at 9.5 g/dL. Histology revealed a lymphomatous proliferation of diffuse architecture, nodular in places, with Hodgkin and Sternberg cells associated with small lymphocytes, histiocytes and eosinophilic polymorphs. Immunohistochemistry showed CD20, PAX5, BCL2, CD5, CD23 and MYC positivity; Ki67 at 10% and cyclin D1, BCL6 and CD10 negativity; CD30 positivity on Hodgkin and Sternberg cells that remained CD20 negative; difficulty interpreting CD15; EBV positivity (EBERs); and CD3 and CD5 positivity on reactive T cells. CD138 and kappa and lambda light chains were non-contributory. The extension work-up classified the patient as Ann Arbor stage III B with a Hasenclever score of 3/7. This case illustrates the difficulties in diagnosing HvRS in our countries, where the number of haematopathologists is insufficient and the technical facilities are limited.

霍奇金变异里希特综合征(HvRS)是一种不常见的并发症,发生率为淋巴细胞淋巴瘤/慢性淋巴细胞白血病患者的 1%。我们报告了一例在撒哈拉以南非洲确诊的 HvRS 病例。一名 63 岁的患者因入院前腹部肿块演变 5 年而就诊。病史显示患者盗汗、3 个月内体重下降 13%,并伴有持续性瘙痒。检查发现双侧颈部腋窝和腹股沟大腺病、无痛性腹胀、瘙痒性病变和WHO 2 PS。血细胞计数显示贫血,为 9.5 g/dL。组织学显示淋巴瘤增生呈弥漫性结构,局部呈结节状,有霍奇金细胞和斯特恩伯格细胞,伴有小淋巴细胞、组织细胞和嗜酸性多形性细胞。免疫组化显示 CD20、PAX5、BCL2、CD5、CD23 和 MYC 阳性;Ki67 为 10%,细胞周期蛋白 D1、BCL6 和 CD10 阴性;霍奇金和斯特恩伯格细胞 CD30 阳性,但 CD20 仍然阴性;CD15 难以解释;EBV 阳性(EBERs);反应性 T 细胞 CD3 和 CD5 阳性。CD138以及kappa和lambda轻链无贡献。延伸检查结果将患者归类为Ann Arbor III B期,Hasenclever评分为3/7。该病例说明了在我们国家诊断 HvRS 的困难,因为那里的血液病理学家人数不足,技术设施有限。
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引用次数: 0
Investigation of Delayed Transfusion Reactions in Sickle Cell Disease Patients Polytransfused in the Brazilian Amazon. 巴西亚马逊地区接受多输血的镰状细胞病患者延迟输血反应调查。
IF 1.1 Q4 HEMATOLOGY Pub Date : 2024-08-01 DOI: 10.3390/hematolrep16030049
Lorena Alves Santos, Anne Cristine Gomes de Almeida, Andrea Monteiro Tarragô, Nina Rosa Gonçalves da Silva, Juliana Nascimento Vitoriano da Silva, Mônica Moura de Souza, Monik Oney Oliveira Nascimento, Marcelo Reis do Nascimento, Ana Caroline Dos Santos Castro, Cinthia Xerez de Albuquerque, Evilázio Cunha Cardoso, José Pereira Moura Neto, Sérgio Roberto Lopes Albuquerque

Background: Sickle cell disease (SCD) affects approximately 100,000 people in the United States and millions worldwide, with the highest prevalence of 70% of SCD being found in individuals of African ethnicity. Delayed hemolytic, alloimmunization, and anamnestic transfusion reactions in multiple transfusion patients need to be investigated and managed to avoid a worsening of the patient's clinical status.

Objective: This paper aims to investigate delayed transfusion reactions in SCD patients who were polytransfused in the Brazilian Amazon.

Material and methods: The clinical and laboratory indicators of SCD patients with more than four transfusions were investigated. The patients were treated at the Fundação Hospitalar de Hematologia e Hemoterapia do Estado do Amazonas, Brazil.

Results: A total of 44 polytransfused patients with SCD were followed. Regarding Rh phenotype, it was possible to observe a frequency of 26.6% (12) patients with the RZRZ (DCE/DCE) phenotype, in addition to 4.5% (two) patients with RH and RHCE variants. It was also possible to observe 20.5% (nine) patients with an alloimmunization reaction, who presented the following alloantibodies: anti-RhD, anti-E, anti-K, anti-Jkb, anti-N, anti-S, and anti-Dia, two of which are unidentified. Of these, four (44.4%) patients also presented autoantibodies, anti-e, and three unidentified antibodies, and four (44.4%) patients presented an anamnestic reaction, with anti-RhD, K, and Jkb antibodies. Of the 44 patients monitored, 54.4% (24) had clinical and laboratory indicators of a delayed hemolytic reaction.

Conclusion: Delayed transfusion reactions, often neglected, occur frequently. Therefore, transfusions need to be monitored for at least 28 days, with medical investigation of clinical and laboratory indicators to make greater use of this therapeutic resource.

背景:镰状细胞病(SCD)影响着美国约 10 万人和全球数百万人,其中非洲裔患者的发病率最高,达 70%。需要对多次输血患者的延迟溶血、同种免疫和无症状输血反应进行调查和处理,以避免患者的临床状况恶化:本文旨在调查巴西亚马逊地区多次输血的 SCD 患者的延迟输血反应:对输血超过四次的 SCD 患者的临床和实验室指标进行了调查。这些患者均在巴西亚马孙州血液学和血液治疗基金会医院接受治疗:结果:共跟踪调查了44名多次输血的SCD患者。在Rh表型方面,除了4.5%(2例)的患者具有RH和RHCE变异外,还发现26.6%(12例)的患者具有RZRZ(DCE/DCE)表型。此外,还观察到 20.5%(9 名)的患者有同种免疫反应,他们出现了以下同种抗体:抗 RhD、抗 E、抗 K、抗 Jkb、抗 N、抗 S 和抗 Dia,其中有两种尚未确定。其中,4 名患者(44.4%)还出现了自身抗体、抗 E 和 3 种不明抗体,4 名患者(44.4%)出现了抗 RhD、抗 K 和抗 Jkb 的变态反应。在接受监测的 44 名患者中,54.4%(24 人)有延迟溶血反应的临床和实验室指标:结论:延迟性输血反应常常被忽视,但却经常发生。因此,需要对输血进行至少 28 天的监测,并对临床和实验室指标进行医学调查,以便更好地利用这一治疗资源。
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引用次数: 0
Persistently High Platelet Factor 4 Levels in an Adolescent with Recurrent Late Thrombotic Complications after SARS-CoV-2 mRNA Vaccination. 一名接种 SARS-CoV-2 mRNA 疫苗后反复出现晚期血栓并发症的青少年体内血小板因子 4 水平持续偏高。
IF 1.1 Q4 HEMATOLOGY Pub Date : 2024-07-29 DOI: 10.3390/hematolrep16030048
Yoichi Haga, Akira Ohara, Tsuneyoshi Yakuwa, Akari Yamashita, Midori Udo, Masaki Matsuoka, Hiroshi Ohara, Atsushi Yasumoto, Hiroyuki Takahashi

Thrombosis after severe acute respiratory syndrome coronavirus 2 vaccination is a serious complication in patients with a thrombophilic predisposition. Herein, we present a 17-year-old female who had underlying antiphospholipid syndrome (APS) and developed deep vein thrombosis (DVT) 6 months after her second BNT162b2 vaccine dose. Although she had no family history of thrombosis, she had previously developed DVT at 6 years of age, with thrombus formation in the right common iliac vein and the inferior vena cava, along with concomitant left pulmonary infarction. The patient had received anticoagulant therapy for 6 years after DVT onset, with subsequent treatment cessation for 5 years without recurrence. She received the BNT162b2 vaccine at 17 years of age, 1 week before a routine outpatient visit. Platelet factor 4 elevation was detected 14 days after the first vaccination, persisting for 5 months without thrombotic symptoms. Six months after the second vaccine dose, the DVT recurred and was treated with a direct oral anticoagulant. The vaccine was hypothesized to exacerbate the patient's APS by activating coagulation. Platelet factor 4 levels may indicate coagulation status. When patients predisposed to thrombosis are vaccinated, coagulation status and platelet activation markers should be monitored to prevent DVT development.

接种严重急性呼吸系统综合征冠状病毒 2 疫苗后出现血栓是有血栓倾向患者的严重并发症。在此,我们介绍了一名患有潜在抗磷脂综合征(APS)的 17 岁女性,她在接种第二剂 BNT162b2 疫苗 6 个月后出现了深静脉血栓(DVT)。虽然她没有血栓形成的家族史,但她在 6 岁时曾患过深静脉血栓,右髂总静脉和下腔静脉有血栓形成,并伴有左肺梗塞。该患者在深静脉血栓形成后接受了 6 年的抗凝治疗,随后停止治疗 5 年,未再复发。17 岁时,她在常规门诊就诊前一周接种了 BNT162b2 疫苗。第一次接种疫苗 14 天后发现血小板因子 4 升高,持续 5 个月后未出现血栓症状。第二次接种疫苗 6 个月后,深静脉血栓复发,接受了直接口服抗凝剂治疗。据推测,疫苗激活了凝血功能,从而加重了患者的 APS。血小板因子 4 水平可显示凝血状态。有血栓形成倾向的患者接种疫苗后,应监测凝血状态和血小板活化标志物,以防止深静脉血栓形成。
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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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Hematology Reports
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