Disseminated disease

播散性疾病
  • 文章类型: Case Reports
    涉及女性妇科的原发性或继发性非霍奇金淋巴瘤(NHL)很少见。T细胞亚型更罕见,预示预后更差。我们介绍了一例23岁女性,表现为宫颈肿块,伴有体质症状和异常阴道出血。免疫组织化学研究显示存在弥漫性T细胞非霍奇金淋巴瘤,为间变性淋巴瘤激酶(ALK)阳性。患者在诊断后一年疾病复发后最初和再次对全身化疗表现出完全反应。据我们所知,这是首例ALK阳性T细胞淋巴瘤继发子宫和子宫颈受累;此前发表的所有妇科组织学亚型病例均描述了阴道的原发疾病.该病例强调了在评估宫颈肿瘤活检以做出适当诊断并指导全身治疗时,包括T细胞和B细胞标志物在内的免疫组织化学研究的重要性。
    Primary or secondary non-Hodgkin lymphomas (NHLs) involving the female gynecologic tract are rare. T-cell subtypes are further rare and portend a worse prognosis. We present a case of a 23-year-old female presenting with a cervical mass accompanied by constitutional symptoms and abnormal vaginal bleeding. Immunohistochemistry studies revealed the presence of disseminated T-cell non-Hodgkin lymphoma that was anaplastic lymphoma kinase (ALK)-positive. The patient demonstrated a complete response to systemic chemotherapy initially and again after the relapse of the disease one year after diagnosis. To our knowledge, this is the first case of an ALK-positive T-cell lymphoma with secondary involvement of the uterus and cervix; all previously published cases of this histologic subtype in the gynecologic tract describe primary disease of the vagina. This case emphasizes the importance of immunohistochemistry studies inclusive of T-cell and B-cell markers when evaluating biopsies from cervical tumors to render the appropriate diagnosis and guide systemic therapy.
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  • 文章类型: Case Reports
    组织胞浆菌病是由荚膜组织胞浆菌病引起的真菌病,富含氮的土壤地区特有的双态真菌,就像被鸟和蝙蝠粪便污染的那个。免疫反应不足的患者尤其有发生侵袭性感染的风险,如播散性组织胞浆菌病,继发性免疫缺陷可能是营养不良的结果。该病例报告介绍了一名15个月大的营养不良男婴,并伴有播散性组织胞浆菌病的体征和症状。包括发烧,萎靡不振,减肥,咳嗽,和腹泻。婴儿来自组织胞浆菌病流行的地理区域,他是一个文化群体的成员,组织胞浆菌病的患病率高于普通人群。在体检时,肝脾肿大,淋巴结病,发现了肺部的裂纹,这在大多数组织胞浆菌病患者中很常见。诊断荚膜嗜血杆菌感染的重点是抗原检测,但是标准标准是通过实验室培养从身体标本中分离生物体。组织学诊断对于快速诊断尤其有用。儿科人群中播散性组织胞浆菌病的治疗包括脱氧胆酸两性霉素B四至六周,然后伊曲康唑完成总共三个月的治疗。尽管涉及多个器官系统,该患者在完成两性霉素B治疗1个月并解决营养不良问题后恢复满意.
    Histoplasmosis is a mycosis caused by Histoplasma capsulatum, a dimorphic fungus endemic to areas with nitrogen-rich soil, like the one contaminated with bird and bat excrement. Patients with a deficient immune response are especially at risk for developing invasive infections, such as disseminated histoplasmosis, and secondary immunodeficiency can be a consequence of malnutrition. This case report presents a 15-month-old male infant with malnutrition who presented with signs and symptoms of disseminated histoplasmosis, including fever, malaise, weight loss, cough, and diarrhea. The infant came from a geographic area where histoplasmosis is endemic, and he was a member of a cultural group with a higher prevalence of histoplasmosis than the general population. On physical examination, hepatosplenomegaly, lymphadenopathy, and lung crackles were found, which are common in most patients with histoplasmosis. The keystone of diagnosis of H. capsulatum infection is antigen detection, but the criterion standard is isolation of the organism from body specimens through laboratory culture. Histological diagnosis is especially useful for rapid diagnosis. Treatment of disseminated histoplasmosis in the pediatric population consists of deoxycholate amphotericin B for four to six weeks followed by itraconazole to complete a total of three months of treatment. Despite the involvement of multiple organ systems, the patient recovered satisfactorily after the completion of amphotericin B treatment for one month and the resolution of his malnourishment.
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  • 文章类型: Case Reports
    我们的病例报告证实,有免疫能力的患者在猴痘感染三周后肾上腺功能不全。因为猴痘没有特殊的治疗方法,类固醇替代疗法仍然是治疗肾上腺功能不全的基石.通过在我们的病例报告中强调早期识别和追踪的重要性,我们为不断增长的猴痘知识做出贡献,并强调识别肾上腺功能不全和脑膜脑炎等并发症的重要性,促使一个重要的早期干预,可以帮助有效地管理这些并发症,可能改善患者预后。此外,通过追踪猴痘病例了解疾病进展提供了关于疾病自然史的有价值的信息,包括症状的时间线和顺序,相关并发症,和结果,以避免与我们缺乏有效知识或对疾病或暴露与靶器官影响之间的相关并发症的错误评估相关的任何小机会。
    Our case report confirms adrenal insufficiency following three weeks of monkeypox infection in an immunocompetent patient. As there is no specific treatment for monkeypox, steroid replacement therapy remains the cornerstone of managing adrenal insufficiency. By highlighting the importance of early identification and tracing in our case report, we contribute to the growing body of knowledge on monkeypox and emphasize the importance of identifying adrenal insufficiency and other complications like meningoencephalitis, prompting a significant early intervention that can help manage these complications effectively, potentially improving patient outcomes. In addition, understanding disease progression by tracing monkeypox cases provides valuable information on the disease\'s natural history, including the timeline and sequence of symptoms, associated complications, and outcomes, to avoid any small chance related to our lack of validated knowledge or incorrect assessment of the disease or the associated complications between an exposure and an effect in the target organs.
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  • 文章类型: Case Reports
    芽生菌病是一种真菌传染病,可发生在免疫功能低下和免疫功能正常的人群在北美流行。在日本没有以前的报道。一名没有相关病史的26岁日本女性患者八个月前在当地诊所出现间歇性左背痛和左上肺野异常阴影。她被转诊到我们医院接受进一步的评估和治疗。病人目前住在日本,但直到两年前在纽约呆了几年,佛蒙特州和加州。胸部计算机断层扫描显示30毫米的肿块,左肺心尖有腔。经支气管活检获得的标本显示高碘酸希夫染色(PAS)阳性和Grocott阳性酵母样真菌散布在肉芽肿中,没有恶性发现,最初的病理并没有导致明确的诊断。由于多个皮下脓肿的发作,她在经验上开始使用氟康唑,并被转诊到医学真菌学研究中心。尽管抗体测试不能诊断这种疾病,根据医学真菌学研究中心的皮肤和肺组织的病理学,怀疑有芽生菌病,通过对rRNA区域的ITS分析鉴定了皮肤芽胞菌。氟康唑治疗后症状和CT表现逐渐好转。我们报道了日本首例肺和皮肤受累的胚生菌病。随着海外旅客人数预计将继续增加,我们想强调旅行历史采访和芽生菌病信息的重要性。
    Blastomycosis is a fungal infectious disease that can occur in both immunocompromised and immunocompetent populations endemic in North America, with no previous reports in Japan. A 26-year-old Japanese female patient with no relevant medical history presented intermittent left back pain and an abnormal shadow in the left upper lung field eight months ago at a local clinic. She was referred to our hospital for further evaluation and treatment. The patient currently lives in Japan, but until two years ago had spent several years in New York, Vermont and California. Chest computed tomography revealed a 30 mm mass with a cavity in the left pulmonary apex. The specimens obtained by transbronchial biopsy showed periodic acid-Schiff stain (PAS)-positive and Grocott-positive yeast-like fungi scattered among the granulomas, with no malignant findings, and the initial pathology did not lead to a definitive diagnosis. She was empirically started on fluconazole because of onset of multiple subcutaneous abscesses and was referred to the Medical Mycology Research Center. Although antibody tests could not diagnose the disease, blastomycosis was suspected based on the pathology of the skin and lung tissue at the Medical Mycology Research Center, and Blastomyces dermatitidis was identified by ITS analysis of the rRNA region. Her symptoms and CT findings gradually improved with fluconazole. We reported the first Japanese case of blastomycosis with pulmonary and cutaneous involvement in Japan. As the number of overseas travelers is expected to continue increasing, we would like to emphasize the importance of travel history interviews and information of blastomycosis.
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  • 文章类型: Case Reports
    组织胞浆菌病是由双态物种组织胞浆菌引起的罕见真菌感染(H。)荚膜,在美国中西部和中部发现。在俄亥俄州和密西西比河流域以外的其他地区观察到荚膜嗜血杆菌感染。包括墨西哥和中美洲和南美洲。在拉丁美洲,越来越多的人免疫缺陷病毒(HIV)免疫受损患者中发生这种疾病的报道。本病例报告详述了一名免疫受损患者播散性组织胞浆菌病的临床发现,新诊断为获得性免疫缺陷综合征(AIDS),最初出现来源不明的脓毒症。本病例报告的重点是详细的病史记录指导对适当调查和识别感染源以及在门诊和住院环境中传播的组织胞浆菌病的管理具有重要意义。
    Histoplasmosis is a rare fungal infection caused by the dimorphic species Histoplasma (H.) capsulatum, found in the Midwest and Central United States. Infection with H. capsulatum is observed in other regions beyond the Ohio and Mississippi River valley, including Mexico and Central and South America. There have been increasing reports of the disease occurring in Latin America in immunocompromised patients with human immunodeficiency virus (HIV). This case report details clinical findings of disseminated histoplasmosis in an immunocompromised patient, newly diagnosed with acquired immunodeficiency syndrome (AIDS) and initially presenting with sepsis of unclear source. The focus of this case report is the significance of detailed history-taking guiding for an appropriate investigation and recognition of the infectious source and giving insight into the management of disseminated histoplasmosis in the outpatient and inpatient settings.
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  • 文章类型: Case Reports
    颅骨和颅底可以受到各种良性,肿瘤样,和恶性过程。颅骨转移(SM)可能位于颅骨的任何层中,并且在诊断检查期间可能是偶然的或存在神经系统症状。在本研究中,我们讨论了来自各种指标恶性肿瘤的SMs的发生及其无数的临床表现。这项基于数据的研究包括2018年6月至2020年7月之间的骨转移患者。颅骨骨转移的患者被识别,以及主站点的位置,他们的临床表现,并注意到管理战略。在此期间确定了10例颅骨骨转移患者。4例有颅底位置,临床表现为证候。六名患者原发性乳腺癌,三个来自尤因肉瘤,还有一个来自肺癌.管理根据每个患者的主要部位和症状而有所不同。SM,虽然并不罕见,通常是偶然诊断,但在癌症患者中提出了诊断和管理挑战。
    Calvarium and skull base can be affected by a variety of benign, tumor-like, and malignant processes. Skull metastases (SMs) may be located in any layer of the skull and may be incidental or present with neurological symptoms during the diagnostic workup. In the present study, we discuss the occurrence of SMs from various index malignancies and their myriad clinical presentation. This data-based study includes patients of bone metastases between June 2018 and July 2020. Patients with skull bone metastases were recognized, and location of primary site, their clinical presentation, and management strategy were noted. Ten patients with skull bone metastases were identified during this period. Four patients had skull base location with clinical manifestation as syndromes. Six patients had primary from breast cancer, three from Ewing\'s sarcoma, and one from lung cancer. Management varied according to the primary site and symptoms of each patient. SM, though not rare, is often diagnosed incidentally but presents diagnostic and management challenges in the patient with cancer.
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  • 文章类型: Journal Article
    组织胞浆菌病是一种地方性真菌感染,仅限于特定的地理区域。组织胞浆菌属。是导致免疫功能正常和免疫功能低下患者疾病的主要病原体,从单器官(主要影响肺部)感染到危及生命的传播疾病。有关临床流行病学的知识依赖于成人人群的数据;关于儿科人群的患者和疾病特征知之甚少。因此,我们对2000~2019年间已发表的儿童组织胞浆菌病病例进行了结构化审查.对PubMed进行了文献检索,分析了83例病例的流行病学和临床资料。演示时的平均年龄为9.5±5.5岁,51%是女孩。三分之二的儿童免疫功能低下。大多数儿童患有播散性疾病。最常见的临床症状是呼吸道症状,除了非特定的系统特征,包括发烧,肌痛,疲劳和体重减轻。死亡率为11%。组织胞浆菌病影响任何年龄的儿童。免疫功能低下是严重和播散性疾病的危险因素。缺乏特定的呈现特征会导致漏报和诊断延迟。为了提高儿童对组织胞浆菌病的认识和预后,增强意识和监控系统是必要的。
    Histoplasmosis is an endemic fungal infection that is confined to specific geographical regions. Histoplasma spp. are primary pathogens that cause disease in both immunocompetent and immunocompromised patients, ranging from a single-organ (mostly affecting the lungs) infection to life-threatening disseminated disease. Knowledge about the clinical epidemiology relies on data from adult populations; little is known about the patient and disease characteristics in the paediatric population. Therefore, a structured review of published cases of paediatric histoplasmosis between 2000 and 2019 was performed. A literature search of PubMed was conducted and the epidemiological and clinical data from 83 cases were analysed. The mean age at presentation was 9.5 ± 5.5 years, and 51% were girls. Two-thirds of the children were immunocompromised. The majority of children presented with disseminated disease. The most frequently observed clinical symptoms were respiratory symptoms, alongside non-specific systemic features, including fever, myalgia, fatigue and weight loss. The mortality rate was 11%. Histoplasmosis affects children of any age. Being immunocompromised is a risk factor for severe and disseminated disease. The lack of specific presenting features leads to underreporting and delay in diagnosis. To improve the recognition and outcome of histoplasmosis in childhood, increased awareness and surveillance systems are warranted.
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  • 文章类型: Journal Article
    In this paper, we present a multicentre record-based descriptive study used to estimate the incidence and characterize the spectrum of confirmed bacille Calmette-Guérin (BCG) vaccine-related disease among children in Oman. This study included all children (age ≤ 14 years) who had culture and/or polymerase chain reaction (PCR)-confirmed BCG disease from January 2006 to December 2018, as identified from Central Public Health Laboratory data and International Classification of Diseases coding of an electronic patient information system. In total, 88 children confirmed to have BCG disease were included in the study, making an average incidence of 9.2 cases per 100,000 vaccinated neonates. The males comprised 65.9%, Omanis 93.2%, and the median age of presentation was 4 months in children with BCG disease. The most common type of disease was BCG abscesses (72.4%). Children with immunodeficiency and those presenting within 6 months were found to have a more severe and disseminated disease. In total, 28 children had immunodeficiency. The age of presentation and type of BCG disease was significantly associated with immunodeficiency status. The majority of cases required therapy (both medical and surgical) and recovered well. The incidence of laboratory-confirmed BCG vaccine-related disease was low in Oman supporting continuing the use of the BCG vaccination practice at birth.
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  • 文章类型: Journal Article
    乳腺癌是全球最常见的女性癌症,约12%的患者最终发展为转移性疾病。严重的,转移性乳腺癌的有效治疗方案有限.最近,血管性血友病因子(VWF),一种止血血浆糖蛋白,已被证明在肿瘤进展和转移中起重要作用。在乳腺癌中,据报道,与良性疾病和健康对照相比,恶性疾病患者的血浆VWF水平显着升高,在患有播散性疾病的患者中出现了更大的增长。VWF之间的直接交互,肿瘤细胞,血小板和内皮细胞可能促进血源性播散,从而形成转移灶。有趣的是,转移性疾病患者的VWF多聚体异常大.该观察结果被认为是VWF切割蛋白酶活性功能失调或缺乏的结果。ADAMTS-13活性,然后可以调节转移过程中的血小板-肿瘤粘附相互作用。在这次审查中,我们概述了VWF在协调乳腺癌播散的病理过程,并提供VWF在介导转移性乳腺癌中的作用的支持证据。
    Breast cancer is the most common female cancer globally, with approximately 12% of patients eventually developing metastatic disease. Critically, limited effective treatment options exist for metastatic breast cancer. Recently, von Willebrand factor (VWF), a haemostatic plasma glycoprotein, has been shown to play an important role in tumour progression and metastasis. In breast cancer, a significant rise in the plasma levels of VWF has been reported in patients with malignant disease compared to benign conditions and healthy controls, with an even greater increase seen in patients with disseminated disease. Direct interactions between VWF, tumour cells, platelets and endothelial cells may promote haematogenous dissemination and thus the formation of metastatic foci. Intriguingly, patients with metastatic disease have unusually large VWF multimers. This observation has been proposed to be a result of a dysfunctional or deficiency of VWF-cleaving protease activity, ADAMTS-13 activity, which may then regulate the platelet-tumour adhesive interactions in the metastatic process. In this review, we provide an overview of VWF in orchestrating the pathological process of breast cancer dissemination, and provide supporting evidence of the role of VWF in mediating metastatic breast cancer.
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  • 文章类型: Journal Article
    BACKGROUND: Determining the extent of cryptococcal disease (CD) is key to therapeutic management. Treatment with fluconazole is only recommended for localised pulmonary disease. Induction therapy with amphotericin B (AmB) and flucytosine is recommended for disease at other sites, irrespective of central nervous system (CNS) involvement, but this is not often followed in patients without meningitis. In this study, we compared treatment and mortality between patients with CD of the CNS and other extrapulmonary (OE) sites.
    METHODS: This is a retrospective, single-centre study of all hospitalised patients with nonpulmonary cryptococcal infection from 2002 to 2015 who underwent lumbar puncture. Demographics, predisposing factors, comorbidities, clinical presentation, laboratory values, antifungal treatment and mortality data were collected to evaluate 90-day mortality and treatment differences between patients with OE and CNS CD. Survival analysis was performed using multivariable Cox regression analysis.
    RESULTS: Of 193 patients analysed, 143 (74%) had CNS CD and 50 (26%) had OE CD. Ninety-day mortality was 23% and similar between the OE and CNS CD groups (22% vs 23%, p = .9). In the comorbidity-adjusted multivariable Cox regression model, mortality risk was similar in the OE and CNS groups. Fewer patients with OE CD received induction therapy with AmB and flucytosine compared to those with CNS disease (28% vs 71.3%, p < .001).
    CONCLUSIONS: Patients with OE CD had similar 90-day mortality compared to those with CNS disease. Despite current guideline recommendations, patients with OE disease were less likely to receive appropriate induction therapy with AmB and flucytosine compared to patients with CNS disease.
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