Haematology (incl blood transfusion)

血液学 ( 包括输血 )
  • 文章类型: Case Reports
    斑疹伤寒,流行的热带感染,有时可能表现为不寻常的并发症。这里,我们介绍了一个年轻人的案例,他在过去的三天里发烧,并从那天早上开始出现深色尿液。在调查中,我们发现了血管内溶血性贫血.通过细致的检查,在他的右臀部发现了一个黑色坏死病变(焦痂),斑疹伤寒感染的病理标志。用口服多西环素100mg开始治疗,每天两次。随后,通过ELISA和焦痂组织的PCR分析,斑疹伤寒IgM阳性结果证实了斑疹伤寒的诊断。患者对口服多西环素反应良好,在接下来的几天内症状缓解。此病例突出了与斑疹伤寒感染相关的严重血管内溶血。
    Scrub typhus, a prevalent tropical infection, may sometimes manifest with unusual complications. Here, we present the case of a young man who was admitted to our facility with a fever for the past 3 days and passage of dark-coloured urine since that morning. On investigation, we identified intravascular haemolytic anaemia. Through meticulous examination, a black necrotic lesion (eschar) was discovered on his right buttock, a pathognomonic sign of scrub typhus infection. Treatment was initiated with oral doxycycline 100 mg two times a day. Subsequently, diagnosis of scrub typhus was confirmed through positive results from scrub typhus IgM via ELISA and PCR analysis from the eschar tissue. The patient responded well to oral doxycycline and his symptoms resolved within the next few days. This case highlights severe intravascular haemolysis associated with scrub typhus infection.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Case Reports
    弥漫性大B细胞淋巴瘤(DLBCL)的结外受累定义为淋巴结外疾病,最多可发生在三分之一的患者中。尽管多器官结外受累很少见。这里,我们描述了一个患者出现广泛转移性病变的病例,包括肺的受累,腮腺,乳房,胰腺,股骨和多个软组织肿块,最初担心原发性乳腺恶性肿瘤。乳腺病理和影像学与三重表达者一致,双重打击IV期高级别B细胞淋巴瘤,广泛的结外受累。结外受累是与高治疗失败率相关的不良预后因素,和靶向CD19的新疗法目前正在研究复发和难治性DLBCL。结外疾病是一种复杂的实体,几乎可以涉及任何器官系统,应考虑新的恶性肿瘤表现。
    Extranodal involvement in diffuse large B-cell lymphoma (DLBCL) is defined as disease outside of the lymph nodes and occurs in up to one-third of patients, though multiorgan extranodal involvement is rare. Here, we describe a case of a patient presenting with widely metastatic lesions, including involvement of the lung, parotid gland, breast, pancreas, femur and multiple soft tissue masses, with initial concern for primary breast malignancy. Breast pathology and imaging were consistent with triple-expressor, double-hit stage IV high-grade B-cell lymphoma with extensive extranodal involvement. Extranodal involvement is a poor prognostic factor associated with high rates of treatment failure, and novel therapies targeting CD19 are currently being studied for relapsed and refractory DLBCL. Extranodal disease is a complex entity that can involve virtually any organ system and should be considered for new presentations of malignancy.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Case Reports
    我们描述了一名30多岁的孕妇双侧肺静脉血栓形成的不寻常病例,在妊娠34周时出现突然发作的胸痛症状,呼吸急促和近乎晕厥的发作。患者接受依诺肝素治疗,临床和血流动力学恢复良好。
    We describe an unusual case of bilateral pulmonary venous thrombosis in a pregnant woman in her mid 30s, who presented at 34 weeks of gestation with symptoms of sudden onset chest pain, shortness of breath and near syncope attacks. The patient was treated with enoxaparin and made an excellent clinical and hemodynamic recovery.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Case Reports
    多发性骨髓瘤是一种罕见的血液恶性肿瘤,其特征是骨髓内浆细胞的克隆增殖。典型的表现包括骨痛,血清和尿液中的疲劳和单克隆蛋白升高。少于1%的病例出现骨髓瘤性胸腔积液,严重的并发症表明晚期疾病和非常差的预后。这里,我们介绍了一个新诊断为多发性骨髓瘤并伴有双侧骨髓瘤性胸腔积液的女性病例。这个病例强调了多发性骨髓瘤的不同临床谱,及时诊断的意义和与骨髓瘤性胸腔积液相关的威胁意义。
    Multiple myeloma is a rare haematological malignancy characterised by the clonal proliferation of plasma cells within the bone marrow. Typical manifestations include bone pain, fatigue and monoclonal protein elevation in serum and urine. Less than 1% of cases develop myelomatous pleural effusion, a severe complication indicative of advanced disease and a very poor prognosis.Here, we present a case of a woman with a new diagnosis of multiple myeloma complicated by bilateral myelomatous pleural effusions as the initial presentation. This case underscores the diverse clinical spectrum of multiple myeloma, the significance of timely diagnosis and the threatening implications associated with myelomatous pleural effusions.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 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.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Case Reports
    Babesiosis是tick传播的寄生虫感染,可导致各种血液学并发症。此病例报告讨论了一名患有严重巴贝西虫病的患者,并伴有巴贝西虫病相关的溶血性尿毒综合征的非正统表现。这里讨论的是患者的临床过程和采用的管理策略,重点是早期识别和治疗严重的Babesiosis背景下的肾功能衰竭。巴贝斯虫的血液学表现很常见,疾病的严重程度取决于寄生虫的负荷。虽然已经提出了针对严重病例的治疗选择,例如红细胞交换,它们对临床结局的影响有限,在资源有限的环境中可能无法获得.已经提出了使用抗生素的传统管理,但是关于管理独特的表现,例如巴贝西虫病的肾衰竭的讨论有限。因此,了解病理生理学,早期识别和积极的治疗策略可以优化临床结局并降低死亡率.
    Babesiosis is a tick-borne parasitic infection that can result in various haematological complications. This case report discusses a patient with severe Babesiosis complicated by an unorthodox presentation of Babesiosis-associated haemolytic uremic syndrome. Discussed here is the patient\'s clinical course and the management strategies employed, with an emphasis on early recognition and treatment of renal failure in the context of severe Babesiosis. Haematologic manifestations of Babesia are common and the severity of disease is dependent on parasite load. While treatment options such as red blood cell exchange have been proposed for severe cases, their impact on clinical outcomes is limited and they may not be readily available in resource-limited settings. Traditional management using antimicrobials has been proposed but there is limited discussion about managing unique presentations such as renal failure in Babesiosis. Hence, understanding the pathophysiology, early recognition and aggressive treatment strategies can optimise clinical outcomes and reduce mortality.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Case Reports
    我们介绍了一例患有先天性血栓性血小板减少性紫癜的儿童,该儿童发现ADAMTS13基因中具有复合杂合变体,该变体具有新的变体,导致ADAMTS13外显子9-11的大量重复。通过染色体微阵列分析,通过其他分子测试鉴定了该变体。据我们所知,该检测方法以前未用于鉴定ADAMTS13变异体,通过遗传咨询师的参与,可以进行额外的检测.
    We present a case of a child with congenital thrombotic thrombocytopenic purpura found to have a compound heterozygous variant in the ADAMTS13 gene with a novel variant resulting in a large duplication of exons 9-11 of ADAMTS13 This variant was identified through additional molecular testing via a chromosomal microarray analysis. To our knowledge, this assay had not previously been utilised to identify an ADAMTS13 variant and the additional testing was possible through the involvement of a genetic counsellor.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Case Reports
    噬血细胞性淋巴组织细胞增生症(HLH)是一种罕见且危及生命的高炎症综合征,其特征是持续发烧,血细胞减少,肝脾肿大和全身性炎症。继发性HLH可由各种病因引发,包括感染,恶性肿瘤和自身免疫性疾病。我们通过描述一个患有HLH的萨尔瓦多青少年移民的案例来强调HLH诊断和管理的复杂性,新诊断的艾滋病毒,链球菌菌血症和播散性组织胞浆菌病。患者表现为神经和眼部表现,并伴有持续发烧和血细胞减少。他被诊断出患有HLH,除了接受艾滋病毒治疗外,还接受了anakinra的治疗,链球菌菌血症和组织胞浆菌病。患者的HLH在没有皮质类固醇或化疗的情况下消退,被认为是HLH治疗的支柱。该病例强调需要对出现HLH的患者进行多种感染的评估和管理以及个性化管理以实现有利的结果。
    Haemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening hyperinflammatory syndrome characterised by persistent fevers, cytopenia, hepatosplenomegaly and systemic inflammation. Secondary HLH can be triggered by various aetiologies including infections, malignancies and autoimmune conditions. We highlight the complexity of HLH diagnosis and management by describing a case of an adolescent Salvadoran immigrant with HLH, newly diagnosed HIV, Streptococcal bacteraemia and disseminated histoplasmosis. The patient presented with neurological and ocular findings along with persistent fevers and cytopenia. He was diagnosed with HLH and treated with anakinra in addition to receiving treatment for HIV, Streptococcal bacteraemia and histoplasmosis. The patient\'s HLH resolved without corticosteroids or chemotherapy, which are considered the mainstays for HLH treatment. This case underscores the need for the evaluation and management of multiple infections and individualised management in patients presenting with HLH to achieve favourable outcomes.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Case Reports
    获得性因子X(FX)缺乏是AL淀粉样变性的罕见但有据可查的临床特征。由于循环FX吸附到淀粉样原纤维上,FX缺乏症患者可表现出临床上显着的出血素质。这里,我们报道了一个不寻常的病例,一个60多岁的男人出现了6个月的间歇性瘀伤,实验室证明了新的外汇缺陷,游离λ轻链升高用于潜在的AL淀粉样变性和并发的新的腓骨静脉血栓形成。这是关于AL-淀粉样蛋白诱导的FX缺乏症中并发血栓并发症的首次报道。我们讨论了在FX缺乏的情况下,以瘀伤为主要临床症状的AL淀粉样变性的诊断和治疗难题,以及急性深静脉血栓形成的管理。
    Acquired factor X (FX) deficiency is a rare but well-documented clinical feature of AL amyloidosis. Patients with FX deficiency can present with clinically significant bleeding diathesis due to the adsorption of circulating FX to amyloid fibrils. Here, we report an unusual case of a man in his 60s who presented with 6 months of intermittent bruising, labs demonstrating new FX deficiency, elevated free lambda light chains for underlying AL amyloidosis and concurrent new peroneal vein thrombosis. This is the first report of concurrent thrombotic complications in the setting of AL-amyloid-induced FX deficiency. We discuss the diagnostic and therapeutic conundrum of diagnosing AL amyloidosis with bruising as the leading clinical symptom and the management of acute deep vein thrombosis in the setting of FX deficiency.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Journal Article
    一名40多岁的男性患者,因不明原因发烧和临床病理特征而不适数月,可疑为噬血细胞性淋巴组织细胞增多症,并伴有亚急性呼吸急促。CT肺血管造影显示毛玻璃改变,涉及所有肺叶,并具有鼻尖梯度。这些变化,联合长期类固醇暴露治疗肉芽肿性肝炎而不预防肺囊虫,引起对肺囊虫肺炎(PJP)的关注。随后的支气管镜灌洗标本对PJP的PCR呈阳性,患者开始接受适当的治疗。临床和放射学变化最初有所改善,但在完成治疗后,症状和放射学异常复发。二线治疗的再治疗再次导致最初的改善,随后复发并急性恶化。对替代诊断进行了进一步调查,进行了外科肺活检,最终发现免疫抑制相关的EB病毒阳性大B细胞淋巴瘤伴3级淋巴瘤样肉芽肿。
    A male patient in his 40s who had been unwell for months with fever of unknown origin and clinicopathological features suspicious for haemophagocytic lymphohistiocytosis presented to hospital with worsening subacute shortness of breath. CT pulmonary angiogram demonstrated ground glass changes involving all lung lobes with an apicobasal gradient. These changes, combined with long-term steroid exposure for granulomatous hepatitis without pneumocystis prophylaxis, raised concern for pneumocystis jirovecii pneumonia (PJP). A subsequent bronchoscopic lavage specimen was positive on PCR for PJP and the patient was started on appropriate therapy. Clinical and radiological changes initially improved but after completion of therapy, symptoms and radiological abnormalities returned. Retreatment with second-line treatment resulted again in initial improvement followed by relapse with acute deterioration. Further investigations for an alternate diagnosis were made, with a surgical lung biopsy performed finally revealing immunosuppression-related Epstein-Barr virus positive large B cell lymphoma with lymphomatoid granulomatosis of grade 3 pattern.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

公众号