immunomodulators

免疫调节剂
  • 文章类型: Case Reports
    炎症性肠病(IBD)是一种自身免疫性疾病,其特征是影响肠道的慢性炎症。克罗恩病(CD)和溃疡性结肠炎(UC)属于IBD保护伞,需要不同的治疗方法,包括类固醇,免疫调节剂如6-巯基嘌呤(6-MP)和硫唑嘌呤(AZA),和生物制剂。免疫调节剂的长期使用,比如AZA,与发展淋巴瘤的风险升高有关。此病例报告集中于一位77岁的绅士,定期进行胃肠病学监测,接受长期AZA治疗的CD管理。他出现了腭肿胀和慢性急性背痛,诊断为腭部滤泡性淋巴瘤伴转移。对于椎旁病变给予姑息性放疗,患者目前情况稳定。总之,这一病例强调了认识到肿瘤风险增加的重要性,尤其是淋巴瘤,在接受长期免疫调节剂治疗的患者中。它强调需要对病人护理采取警惕和全面的方法,超越传统范式。
    Inflammatory bowel disease (IBD) is an autoimmune disorder marked by chronic inflammation affecting the intestines. Crohn\'s disease (CD) and ulcerative colitis (UC) fall under the IBD umbrella, necessitating diverse treatments, including steroids, immunomodulators like 6-mercaptopurine (6-MP) and azathioprine (AZA), and biological agents. The prolonged use of immunomodulators, such as AZA, is associated with an elevated risk of developing lymphomas. This case report centers on a 77-year-old gentleman regularly monitored by Gastroenterology, undergoing long-term AZA therapy for CD management. He presented with palate swelling and acute-on-chronic back pain, diagnosed with follicular lymphoma in the palate with metastasis. Palliative radiotherapy was administered for the paraspinal lesion, and the patient is currently stable. In conclusion, this case underscores the importance of recognizing the heightened risk of neoplasms, especially lymphomas, in patients undergoing prolonged immunomodulator therapy. It emphasizes the need for a vigilant and comprehensive approach to patient care, transcending conventional paradigms.
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  • 文章类型: Case Reports
    多中心网状组织细胞增生症(MRH)是一种罕见的,与皮肤和关节受累相关的IIb类非朗格汉斯细胞组织细胞增生症。它在高加索女性中在他们的第五到第六十年中更普遍(80%)。患者通常表现出对称性多关节炎和丘疹性皮肤病变的症状和体征。除了皮肤和关节,可能涉及多个器官,如肺(胸腔积液,间质纤维化,肺门淋巴结病),心脏(心包积液,心肌炎),胃肠系统,和泌尿生殖系统(生殖道和肾脏)。心包受累是一种罕见的表现,到目前为止,文献中已经报道了大约三例。我们的病例报告是对文献的宝贵贡献,这有助于临床医生将MRH视为存在心包积液的患者之间的差异之一。我们描述了MRH的特征及其与其他自身免疫性疾病和管理的区别特征。
    Multicentric reticulohistiocytosis (MRH) is a rare, class IIb non-Langerhans cell histiocytosis associated with skin and joint involvement. It is more prevalent (80%) in Caucasian females in their fifth to sixth decade of life. Patients usually demonstrate symptoms and signs of symmetric polyarthritis and papulonodular cutaneous lesions. In addition to skin and joints, multiple organs can be involved, such as the lung (pleural effusion, interstitial fibrosis, hilar lymphadenopathy), heart (pericardial effusion, myocarditis), gastrointestinal system, and urogenital system (genital tract and kidney). Pericardial involvement is a rare manifestation, and around three cases have been reported in the literature so far. Our case report is a valuable contribution to the literature, which aids clinicians in contemplating MRH as one of the differentials among patients presenting with pericardial effusion. We described the characteristics of MRH along with its differentiating features from other autoimmune conditions and management.
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  • 文章类型: Case Reports
    急性早幼粒细胞白血病(APL)是一种独特的,高度可治愈的急性髓系白血病亚型,由于过去几十年的治疗进展,导致高完全缓解率和优异的长期生存率。然而,它仍然与高早期死亡率有关。早期死亡是APL治疗失败的主要原因,主要归因于凝血病,分化综合征,不太常见,传染病事件。及时识别每种并发症在诊断为APL的患者的管理中起着至关重要的作用。2019年冠状病毒传染病(COVID-19)在患者表现出很大的异质性。临床表现从无症状疾病到严重疾病,主要以导致急性呼吸窘迫和多器官衰竭的过度炎症综合征为特征。急性白血病和COVID-19相关的高炎症综合征患者的预后特别差。我们特此报告一例28岁男性患者被诊断为高危APL,出现时伴有严重的相关性凝血病。他根据AIDA方案接受化疗。诱导治疗的第一周并发分化综合征,表现为非感染引起的发热和伴有肺浸润的呼吸窘迫,ATRA停药和皮质治疗后缓解。在治疗的第四周,他的急性呼吸道综合征冠状病毒2(SARS-CoV-2)检测呈阳性,肺部轻微受累。随后几天的临床表现包括心动过速和低血压,与升高的炎症标志物和心脏生物标志物(肌钙蛋白I×58上NV)相关。心血管磁共振成像与心肌炎一致。COVID-19相关性心肌炎用甲基强的松龙成功治疗,静脉注射免疫球蛋白和Anakinra。分化综合征和COVID-19相关心肌炎是两种威胁生命的并发症,对生存率产生不利影响。然而,早期识别和及时开始治疗可以改善临床结果,我们的病人也是如此.
    Acute promyelocytic leukemia (APL) is a unique, highly curable subtype of acute myeloid leukemia, owing to the therapeutic advances of the last decades which led to high complete remission rates and excellent long-term survival. Nevertheless, it remains associated with high early mortality rates. Early death is the major cause of treatment failure in APL and is mainly attributed to coagulopathy, differentiation syndrome, and less commonly, infectious events. Timely recognition of each complication plays a crucial role in the management of patients diagnosed with APL. Coronavirus Infectious Disease 2019 (COVID-19) has shown great heterogeneity in patient presentation. Clinical manifestations range from asymptomatic disease to severe forms, mainly characterized by a hyperinflammatory syndrome leading to acute respiratory distress and multiorgan failure. Patients with acute leukemia and concomitant COVID-19-related hyperinflammatory syndrome have particularly poor outcomes. We hereby report the case of a 28-year-old male patient who was diagnosed with high-risk APL, with severe associated coagulopathy at presentation. He was treated with chemotherapy according to the AIDA regimen. The first week of induction therapy was complicated by a differentiation syndrome manifesting as fever not attributable to infection and respiratory distress with pulmonary infiltrates, resolved after ATRA discontinuation and corticotherapy. On the fourth week of treatment, he tested positive for acute respiratory syndrome coronavirus 2 (SARS-CoV-2) with minor pulmonary involvement. Clinical manifestations over the following days included tachycardia and hypotension, associated with elevated inflammatory markers and cardiac biomarkers (troponin I x58 upper NV). Cardiovascular magnetic resonance imaging was consistent with myocarditis. COVID-19-associated myocarditis was successfully treated with methylprednisolone, intravenous immunoglobulins and Anakinra. Differentiation syndrome and COVID-19-associated myocarditis are two life-threatening complications that adversely impact survival. However, early recognition and prompt treatment initiation can improve clinical outcomes, as was the case of our patient.
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  • 文章类型: Case Reports
    Herpes simplex virus (HSV) encephalitis is a common cause of severe and potentially fatal encephalitis. Autoimmune post-herpes simplex encephalitis (AIPHSE) affects a percentage of patients who developed herpes simplex encephalitis (HSE) and is characterized by the onset of new neurological/psychiatric symptoms and/or worsening of deficits acquired during the herpes infection within a predictable time frame. It is caused by a mechanism not related to HSV, but by autoimmune conditions, and is susceptible to treatment with immunomodulators. Here we describe the case of a 5-year-old boy with AIPHSE who required first- and second-line immunomodulatory treatment, with an adequate course and remission of symptoms.
    La encefalitis por virus herpes simple (VHS) es una causa frecuente de encefalitis grave y potencialmente fatal. La encefalitis autoinmune posherpética (EAPH) afecta a un porcentaje de los pacientes que han presentado encefalitis herpética (EH) y se caracteriza por la aparición de nuevos síntomas neurológico/psiquiátricos, y/o por el empeoramiento de los déficits adquiridos durante la infección viral dentro de un lapso temporal predecible. Se produce por un mecanismo no relacionado con el VHS, sino por fenómenos autoinmunes, y es susceptible de tratamiento con inmunomoduladores. Se presenta el caso de un varón de 5 años de edad con EAPH que requirió tratamiento inmunomodulador, de primera y segunda línea, con buena evolución y remisión de los síntomas.
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  • 文章类型: Case Reports
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  • 接受硫嘌呤治疗的Cohn病(CD)患者患癌症的风险增加。白血病的发生频率低于其他造血系统肿瘤,免疫抑制后慢性粒细胞白血病(CML)的发展尚未得到证实。
    我们描述了一个61岁的女性病例,她在用硫唑嘌呤(AZA)治疗与十二指肠定位相关的回肠克罗恩病8年后发展为CML。我们回顾了目前关于CD之间相互作用的证据,慢性粒细胞白血病和AZA以及AZA治疗患者白血病的潜在潜在潜在机制。
    我们得出结论,CML的发病机制在CD中是多因素的。CD患者中AZA与CML之间的关联性质值得进一步研究。
    BACKGROUND: Patients with Cohn\'s disease (CD) treated with thiopurines are at an increased risk of developing cancer. Leukemias are less frequent than other hematopoietic tumors and the development of Chronic myeloid leukemia (CML) after immunosuppression has not been proven.
    METHODS: We describe the case of a 61-year-old female who developed a CML after 8 years of treatment with azathioprine (AZA) for ileal Crohn\'s disease associated with a duodenal localization. We reviewed the current evidence on the interactions between CD, CML and AZA as well as the potential underlying mechanisms of leukemia in AZA-treated patients.
    CONCLUSIONS: We concluded that the pathogenesis of CML is multifactorial in CD. The nature of the association between AZA and CML in CD patients warrants further investigation.
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  • 文章类型: Case Reports
    快速进展性痴呆(RPD)的诊断非常具有挑战性。有许多疾病属于RPD的类别,从自身免疫原因到神经退行性原因。由于自身免疫性脑炎对免疫调节剂的反应,因此应易于诊断和治疗。然而,对于诸如克雅氏病(CJD)的病症没有可用的治疗。
    在这里,我们介绍了一例抗富含亮氨酸的神经胶质瘤灭活1(LGI1)脑炎,该脑炎仅表现为典型的面臂肌张力障碍发作。在后续行动中,随着肌阵挛性抽搐的发展,患者的认知能力迅速下降,运动状态和最终死亡。神经影像学显示,在液体衰减的倒置恢复(FLAIR)序列上,顶叶和枕骨两个皮质区域存在高强度。脑电图显示弥漫性减慢,偶尔有周期性的尖锐波复合物。因此做出了可能的CJD的诊断。
    模仿CJD或相反的自身免疫性脑炎不是很常见的现象。本案例讨论了这两种情况的临床重叠及其诊断困境。该病例表现为典型的LGI1脑炎,尽管使用免疫调节剂进行了治疗,但其迅速下降,最终被证明是CJD。这在文献中很少描述。
    UNASSIGNED: Diagnosis of rapidly progressive dementia (RPD) is very challenging. There are many conditions that fall into category of RPD ranging from autoimmune causes to neurodegenerative causes. Autoimmune encephalitis should be readily diagnosed and treated because of its response to immunomodulators. However there is no treatment available for conditions like Creutzfeldt-Jakob disease (CJD).
    UNASSIGNED: Here we present a case of anti-leucine-rich glioma inactivated 1 (LGI1) encephalitis who presented with only typical facio-brachial dystonic seizures at presentation. On follow up, patient had a rapid cognitive decline with development of myoclonic jerks, akinetic mute state and ultimately death. Neuroimaging showed presence of hyperintensities in two cortical regions namely parietal and occipital on fluid-attenuated inversion recovery (FLAIR) sequence. Electroencephalogram showed diffuse slowing with occasional periodic sharp wave complexes. Thus a diagnosis of probable CJD was made.
    UNASSIGNED: Autoimmune encephalitis mimicking CJD or vice versa is not a very commonly encountered phenomenon. This case discusses the clinical overlap of these two conditions and its diagnostic dilemmas. This case presented with typical LGI1 encephalitis and in spite of therapy with immunomodulators had a rapid decline and ultimately turned out to be CJD. This has been rarely described in literature.
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  • 文章类型: Case Reports
    复发性口疮性口炎(RAS)是一种以疼痛性口腔溃疡为特征的口腔疾病。虽然这种疾病的临床特征很容易定义,病因尚不清楚。因此,现有的治疗方法在减轻严重程度方面仍然不能令人满意,愈合,和复发率;然而,没有永久和明确的治疗。口疮性口炎的有效治疗是不可用的,可用的治疗方法主要集中在抑制其症状。我们正在报告一例17岁的男孩,他在口腔中有3年的多发性复发性大溃疡病史。左旋咪唑与类固醇已在许多临床试验中用于治疗口疮溃疡,显示出疼痛的改善,不适,愈合时间,和减少溃疡的数量。同样的方法被用来治疗我们的病人,谁表现出了有希望的结果,一年没有复发。左旋咪唑是个保险箱,易于耐受和有前途的药物治疗RAS。
    Recurrent aphthous stomatitis (RAS) is an oral condition characterized by painful oral ulcerations. While the clinical features of this disease are easily defined, the etiology remains unclear. Thus, existing treatments are still unsatisfactory in reducing the severity, healing, and recurrence rate; however, there is no permanent and definitive treatment. Effective treatment for aphthous stomatitis is not available, and those treatments available mainly focus on suppressing its symptoms. We are reporting a case of a 17-year-old boy who presented with a 3-year history of multiple recurrent major ulcers in the oral cavity. Levamisole with steroids has been used in many clinical trials to treat aphthous ulcers, showing an improvement in pain, discomfort, healing time, and reduction in the number of ulcers. The same method was used to treat our patient, who showed promising results, with no recurrence for one year. Levamisole is a safe, easily tolerable and promising drug for the treatment of RAS.
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  • 文章类型: Case Reports
    即使是最有经验的临床医生,坏疽性脓皮病也可能是具有挑战性的诊断。误诊可能导致延误适当的治疗和不必要的清创,从而增加疾病的严重程度。阴茎坏疽性脓皮病(PG)是这种病理过程的罕见表现。我们描述了一名42岁有阴茎骨折史的男性的晚期阴茎PG的诊断检查和成功治疗,该男性表现为伤口愈合延迟和多次泌尿外科手术失败。这个案例表明了保持广泛差异的重要性,包括PG,以避免延误适当的护理。
    Pyoderma gangrenosum can be a challenging diagnosis for even the most experienced clinician. Misdiagnosis can lead to delays in appropriate treatment and unwarranted debridement that can increase the severity of the disease. Penile pyoderma gangrenosum (PG) is a rare presentation of this pathologic process. We describe the diagnostic workup and successful treatment of advanced penile PG in a 42-year-old male with a history of penile fracture who presented with delayed wound healing and multiple unsuccessful urologic surgeries. This case demonstrates the importance of keeping a broad differential, including PG, in order to avoid delays to appropriate care.
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  • 文章类型: Journal Article
    后部可逆性脑病综合征(PRES)是一种临床综合征,可包括头痛,改变了意识,视觉障碍,和癫痫发作,通常与自动调节性脑衰竭和高血压有关。神经成像对诊断至关重要,后部显示白质血管源性水肿。我们介绍了一例66岁的SARS-CoV-2重症肺炎妇女,该妇女发展为后部可逆性脑病综合征,具有典型的临床和放射学表现,在遵循当地指南接受抗白细胞介素治疗(阿纳金拉和托珠单抗)治疗后。我们报告了一例COVID-19病患者的后部可逆性脑病综合征,可能与抗IL-1或抗IL-6有关,提示抗白细胞介素治疗可能引起这种综合征,至少在患有感染和水电解质紊乱等易感疾病的患者中。
    Posterior reversible encephalopathy syndrome (PRES) is a clinical syndrome that can include headache, altered consciousness, visual disturbances, and seizures, usually related to autoregulatory cerebral failure and hypertension. The neuroimaging is essential to diagnosis, showing white matter vasogenic edema in posterior areas. We present a case of a 66-year-old woman with severe pneumonia by SARS-CoV-2 who developed a posterior reversible encephalopathy syndrome with a typical clinical and radiological presentation, after being treated with anti-interleukin treatment (anakinra and tocilizumab) following local guidelines. We report a case of posterior reversible encephalopathy syndrome in a patient with COVID-19 disease, possibly related to anti-IL-1 or anti-IL-6, suggesting that anti-interleukin treatments may cause this syndrome, at least in patients with predisposing conditions such as infections and hydroelectrolytic disorders.
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