Haematology (incl blood transfusion)

血液学 ( 包括输血 )
  • 文章类型: Case Reports
    相对于肝脏病因,高氨血症的非肝脏原因并不常见。一名青春期女性因诊断为非常严重的再生障碍性贫血而入院。在她接受免疫抑制治疗期间,她患上了中性粒细胞减少性小肠结肠炎,假性菌血症和高氨血症。需要间歇性血液透析和高容量连续静脉-静脉血液透析滤过(CVVHDF)的组合来管理高氨血症。尽管进行了彻底的调查,没有肝脏,确定的代谢或遗传病因可以解释高氨血症。高氨血症仅在手术切除发炎的结肠后才解决,随后,她成功地从肾脏支持中断奶。这是一个新的病例报告,涉及非肝脏来源的高氨血症,继发于广泛的结肠炎症,需要在免疫功能低下的患者中进行手术切除。此病例还强调了高容量CVVHDF在增强血液透析治疗严重难治性高氨血症中的作用。
    Non-hepatic causes of hyperammonaemia are uncommon relative to hepatic aetiologies. An adolescent female was admitted to the hospital with a diagnosis of very severe aplastic anaemia. During her treatment with immunosuppressive therapy, she developed neutropenic enterocolitis, pseudomonal bacteraemia and hyperammonaemia. A combination of intermittent haemodialysis and high-volume continuous veno-venous haemodiafiltration (CVVHDF) was required to manage the hyperammonaemia. Despite a thorough investigation, there were no hepatic, metabolic or genetic aetiologies identified that explained the hyperammonaemia. The hyperammonaemia resolved only after the surgical resection of her inflamed colon, following which she was successfully weaned off from the renal support. This is a novel case report of hyperammonaemia of non-hepatic origin secondary to widespread inflammation of the colon requiring surgical resection in an immunocompromised patient. This case also highlights the role of high-volume CVVHDF in augmenting haemodialysis in the management of severe refractory hyperammonaemia.
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  • 文章类型: Case Reports
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  • 文章类型: Journal Article
    我们报告了一名70多岁的男子,他同时在不同医院中出现血清肌酐浓度差异。对这些差异的进一步检查显示血清样品的浊度,因此,怀疑副蛋白干扰引起的试剂反应和假高肌酐。血清蛋白电泳显示少量单克隆γ球蛋白(2.9g/L),这可能与副蛋白干扰有关。在血清中检测到单克隆λ型IgG,导致诊断出意义不明的单克隆丙种球蛋白病。先前的研究表明,血清中含有单克隆IgM或大量IgG(>25g/L)的副蛋白干扰。尽管这种由少量IgG引起的副蛋白干扰的情况很少见,肌酐结果的差异可能是导致浆细胞增殖性疾病诊断的指标。
    We report a man in his 70s who presented with discrepant serum creatinine concentrations in different hospitals at the same time. Further examinations of these discrepancies revealed turbidity of the serum sample and, thus, a reagent reaction and false hypercreatinine caused by paraprotein interference were suspected. Serum protein electrophoresis revealed a small amount of monoclonal γ globulin (2.9 g/L), which may have been involved in paraprotein interference. Monoclonal λ-type IgG was detected in the serum, resulting in a diagnosis of monoclonal gammopathy of undetermined significance. Previous studies indicated paraprotein interference in serum containing monoclonal IgM or a large amount of IgG (> 25 g/L). Although this case of paraprotein interference induced by a small amount of IgG is rare, a discrepancy in creatinine results may be an indicator leading to the diagnosis of plasma cell proliferative diseases.
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  • 文章类型: Case Reports
    多发性骨髓瘤(MM)患者的髓外复发通常与生化反应丧失和骨髓中可测量的残留疾病的出现有关。发烧是MM的不寻常表现。蛋白酶体抑制剂进展的患者髓外复发的治疗,抗CD38单克隆抗体和免疫调节药物具有挑战性,因为获得嵌合抗原受体T细胞和双特异性抗体的途径有限。我们报告了一例复发性MM,表现为发烧和模仿肝细胞癌的肝占位性病变。在这个案例报告中,我们还介绍了我们使用包含Dara-Pom-Benda-Dexa(daratumumab,泊马度胺,地塞米松和苯达莫司汀)用于复发MM。
    Extramedullary relapse in patients with multiple myeloma (MM) is often associated with loss of biochemical response and the appearance of measurable residual disease in the bone marrow. Fever is an unusual presenting manifestation of MM. Treatment of extramedullary relapse in patients progressing on proteasome inhibitors, anti-CD38 monoclonal antibodies and immunomodulatory drugs is challenging, as access to chimeric antigen receptor T-cells and bispecific antibodies is limited. We report a case of relapsed MM who presented with fever and hepatic space-occupying lesion mimicking hepatocellular carcinoma. In this case report, we also present our experience of using a novel combination regimen comprising Dara-Pom-Benda-Dexa (daratumumab, pomalidomide, dexamethasone and bendamustine) for relapsed MM.
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    我们介绍了一例30多岁的男子,表现为ST段抬高型心肌梗死和嗜酸性粒细胞增多。患者接受了血栓抽吸术,最初的超声心动图评估正常。患者2天后出院,但6天后再次住院。超声心动图评估现在显示主动脉瓣上有血栓形成。实验室数据显示嗜酸性粒细胞增多,由于不能排除有器官表现的嗜酸性粒细胞疾病,因此开始接受高剂量糖皮质激素和羟基脲治疗.嗜酸性粒细胞恢复正常,患者再次出院。嗜酸性粒细胞增多和没有感染的组合,风湿性疾病和恶性肿瘤,导致反应性或特发性嗜酸性粒细胞增多综合征是最合理的诊断。在心脏病学和血液学门诊密切监测患者。超声心动图评价,在患者出院后6周进行,显示血栓块大小的显着回归。
    We present a case of a man in his 30s presenting with ST-segment elevation myocardial infarction and eosinophilia. The patient underwent thrombus aspiration and initially echocardiographic evaluation was normal. The patient was discharged after 2 days, but was hospitalised again after 6 days. Echocardiographic evaluation now revealed a thrombus formation on the aortic valve. Laboratory data revealed increasing eosinophilia, and treatment with high-dosage corticosteroids and hydroxyurea was initiated as eosinophilic disease with organ manifestations could not be precluded. Eosinophils normalised and the patient was discharged again. The combination of hypereosinophilia and absence of infection, rheumatological disorders and malignancy, led to reactive or idiopathic hypereosinophilic syndrome being the most plausible diagnoses. The patient was closely monitored in the cardiology and haematology outpatient clinics. Echocardiographic evaluation, performed 6 weeks after the patient was discharged, showed significant regression in the size of the thrombus mass.
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  • 文章类型: Case Reports
    我们介绍了第一例有免疫能力的患者中原发性巨细胞病毒(CMV)感染继发的同时发生肺炎和免疫性血小板减少性紫癜的病例。口服伐更昔洛韦治疗2周成功导致临床完全康复。CMV传统上与免疫功能低下患者和新生儿的感染有关;然而,免疫活性宿主中出现严重CMV感染的证据.重要的是强调CMV感染的广泛临床表现,以防止诊断延迟和相关的发病率和费用。
    We present the first published case of simultaneous pneumonitis and immune thrombocytopenic purpura secondary to primary cytomegalovirus (CMV) infection in an immunocompetent patient. Treatment with oral valganciclovir for 2 weeks successfully led to complete clinical recovery. CMV is traditionally associated with infection in immunocompromised patients and neonates; however, evidence of severe CMV infections in immunocompetent hosts is emerging. It is important to highlight the broad range of clinical presentations of CMV infections to prevent diagnostic delay and associated morbidity and expense.
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  • 文章类型: Case Reports
    一名70多岁的男子尽管诊断为2型糖尿病,但仍有低糖化血红蛋白(HbA1c)值的病史。他的血糖读数在8到15mmol/L之间,但他的HbA1c值低于27mmol/mol。初步调查显示,红细胞寿命减少是导致HbA1c值低且具有误导性的原因。进一步调查显示慢性肝病和脾肿大,脾功能亢进可能是红细胞更新增加的原因。HbA1c估计不再可靠,因此,正在进行的糖尿病护理是在家庭毛细血管血糖监测的指导下进行的。医疗保健提供者和临床实验室人员需要意识到2型糖尿病患者中HbA1c值非常低的可能临床意义。
    A man in his 70s presented with a history of low glycated haemoglobin (HbA1c) values despite a diagnosis of type 2 diabetes. His blood glucose readings ranged between 8 and 15 mmol/L, but his HbA1c values were below 27 mmol/mol. Initial investigations demonstrated evidence of reduced red blood cell lifespan as a cause of misleadingly low HbA1c values. Further investigation revealed chronic liver disease and splenomegaly, with hypersplenism being the probable cause of increased red blood cell turnover. HbA1c estimation was no longer reliable, so ongoing diabetic care was guided by home capillary blood glucose monitoring. Healthcare providers and clinical laboratorians need to be aware of the possible clinical implications of very low HbA1c values in patients with type 2 diabetes.
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  • 文章类型: Case Reports
    我们报告了一例发烧和面部肿胀的年轻人皮下脂膜炎样T细胞淋巴瘤(SPTCL)。他在评估时患有全血细胞减少症和噬血细胞综合征(HPS)。面部和胸壁皮肤穿刺活检的组织病理学检查显示为SPTCL。鉴于相关的HPS,他开始接受类固醇和多药化疗,随后症状有所改善。
    We report a case of subcutaneous panniculitis-like T-cell lymphoma (SPTCL) in a young man presenting with fever and facial swelling. He had pancytopenia and hemophagocytic syndrome (HPS) on evaluation. The histopathological examination of skin punch biopsy from the face and chest wall showed SPTCL. Given the associated HPS, he was started on steroid and multidrug chemotherapy following which he had symptomatic improvement.
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  • 文章类型: Case Reports
    宫颈与急性淋巴细胞白血病(ALL)的关系极为罕见。在这个案例报告中,我们讨论一个20出头的未婚女子,在紧急情况下出现下腹痛和不规则阴道出血1个月。临床检查和影像学检查显示,宫颈大肿块可能是梗阻性尿路病变。关于评估,在外周血流式细胞术中偶然诊断为CALLA阳性前体B细胞ALL.通过宫颈活检和免疫组织化学标记的组织病理学检查证实了B细胞ALL在宫颈肿块中的参与。她的病史没有提示与白血病有关的体征和症状。文献很少,只有少数病例报告宫颈白血病浸润。本病例报告是最罕见的病例,其中发现前B细胞ALL的主要/初始表现为宫颈受累和阻塞性尿路病变,模仿了晚期宫颈恶性肿瘤的特征。
    Involvement of the cervix with acute lymphoblastic leukaemia (ALL) is extremely rare. In this case report, we discuss an unmarried woman in her early 20s, who presented in the emergency with lower abdominal pain and irregular vaginal bleeding for 1 month. Clinical examination and imaging revealed a large cervical mass probably neoplastic with obstructive uropathy. On evaluation, she was diagnosed incidentally with CALLA-positive precursor B cell ALL in peripheral blood flow cytometry. Involvement of B cell ALL in cervical mass was confirmed by histopathological examination of cervical biopsy and immunohistochemistry markers. Her history was not suggestive of signs and symptoms pertaining to leukaemia. Literature is sparse with only a few cases reporting cervical leukaemic infiltration. The present case report is a rarest case where the primary/initial presentation of precursor B cell ALL was seen with cervical involvement and obstructive uropathy mimicking characteristics of advanced cervical malignancy.
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