secondary hemophagocytic lymphohistiocytosis (hlh)

  • 文章类型: Case Reports
    噬血细胞性淋巴组织细胞增生症(HLH)是一种过度免疫激活的侵袭性综合征。它通常发生在儿童身上,主要是在生命的第一年。原发性噬血细胞性淋巴组织细胞增生症更为常见,通常发生在免疫功能低下的患者中。继发性噬血细胞淋巴组织细胞增生症,另一方面,不太常见,尤其是在有免疫能力的患者中。这里,我们打算介绍一个55岁的男性患者,他没有已知的免疫缺陷,出现鼻出血,并被发现患有EB病毒(EBV)诱导的噬血细胞性淋巴组织细胞增生症。
    Hemophagocytic lymphohistiocytosis (HLH) is an aggressive syndrome of excessive immune activation. It usually occurs in children, mainly during the first year of life. Primary hemophagocytic lymphohistiocytosis is more common and usually occurs in immunocompromised patients. Secondary hemophagocytic lymphohistiocytosis, on the other hand, is less common, especially in immunocompetent patients. Here, we intend to present a case of a 55-year-old male patient who had no known immune deficiency, presented with epistaxis, and was found to have Epstein-Barr virus (EBV)-induced hemophagocytic lymphohistiocytosis.
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  • 文章类型: Case Reports
    噬血细胞性淋巴组织细胞增生症(HLH)是一种罕见的疾病,其特征是极端的免疫激活和过度的炎症。据报道,有家族性病例的患者,免疫缺陷,恶性肿瘤,干细胞移植,和病毒病因。该报告描述了一名76岁以前健康的男性中与人类疱疹病毒6(HHV-6)感染相关的获得性HLH。病人因发烧入院,发冷,和腹痛。诊断检查在影像学上显示胆囊壁增厚,关于胆囊炎。患者开始接受脓毒症治疗。进一步的临床恶化导致广泛的感染性检查。发现患者的可溶性IL-2Ra水平升高,做了骨髓活检,这揭示了HLH。脑脊液和血清中HHV-6聚合酶链反应阳性证实了病毒感染。治疗涉及高剂量类固醇的开始,依托泊苷,还有更昔洛韦.尽管有这些干预措施,患者的临床状况恶化,导致舒适措施的实施,病人最终死了.此病例强调了将HHV-6视为有免疫能力的成年人中HLH的潜在原因的重要性。从这个案子来看,我们推断,有必要提高警惕性,以迅速认识和干预这一具有挑战性的状况。
    Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder characterized by extreme immune activation and excessive inflammation. It has been reported in patients with familial cases, immunodeficiencies, malignancies, stem cell transplants, and viral etiologies. This report describes acquired HLH associated with Human herpesvirus-6 (HHV-6) infection in a 76-year-old previously healthy male. The patient was admitted to the hospital due to fever, chills, and abdominal pain. The diagnostic workup revealed gallbladder wall thickening on imaging, concerning for cholecystitis. The patient was started on treatment for sepsis. Further clinical deterioration led to an extensive infectious workup. The patient was found to have elevated soluble IL-2Ra levels, and a bone marrow biopsy was performed, which revealed HLH. A positive HHV-6 polymerase chain reaction in the cerebrospinal fluid and serum confirmed the viral infection. Treatment involved the initiation of high-dose steroids, etoposide, and ganciclovir. Despite these interventions, the patient\'s clinical status worsened, leading to the implementation of comfort measures, and the patient eventually died. This case underscores the importance of considering HHV-6 as a potential cause of HLH in immunocompetent adults. From this case, we infer that a heightened level of vigilance is necessary to recognize and intervene in this challenging condition promptly.
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  • 文章类型: Case Reports
    噬血细胞淋巴组织细胞增多症(HLH)是一种罕见的,危及生命的高炎症综合征,其特征是免疫系统的深度激活。在成年人中,大多数HLH病例是由于潜在的病理-如感染,恶性肿瘤,或自身免疫性疾病。这是一种可以进展到快速临床恶化并且难以诊断的疾病。然而,不管病因,大多数HLH患者从治疗中获益。本文强调了在实践中诊断和管理这种疾病所涉及的挑战,强调有多年轻,以前健康的年轻人可能处于危重状态。
    Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hyperinflammatory syndrome characterized by profound immune system activation. In adults, most cases of HLH are due to an underlying pathology- such as infection, malignancy, or autoimmune disease. It is a disease that can progress to rapid clinical deterioration and be difficult to diagnose. Nevertheless, regardless of etiology, most patients with HLH benefit from treatment. This paper highlights the challenges involved in diagnosing and managing this condition in practice, with an emphasis on how young, previously healthy young adults can present in a critically ill state.
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  • 文章类型: Case Reports
    噬血细胞性淋巴组织细胞增生症(HLH)是一种罕见的疾病,由于过度的巨噬细胞激活和细胞因子产生,可能是致命的。它可以是原发性(家族性/遗传性)或继发性。它与感染有关,恶性肿瘤,以及风湿病和免疫缺陷疾病。我们报告了一名患有镰状细胞性贫血的中年女性患者,该患者出现COVID-19感染,引发血管闭塞危象并导致HLH。她先前有高铁蛋白水平和血细胞减少症,使诊断更具挑战性。高度怀疑和及时治疗对于预防不良后果至关重要。
    Hemophagocytic lymphohistiocytosis (HLH) is an uncommon condition that can be fatal due to overwhelming macrophage activation and cytokine production. It can be primary (familial/genetic) or secondary. It is associated with infections, malignancies, and rheumatologic and immunodeficiency disorders. We report a middle-aged female patient with sickle cell anemia who presented with COVID-19 infection that triggered a vaso-occlusive crisis and resulted in HLH. She had preexisting high ferritin levels and cytopenias, making the diagnosis more challenging. A high index of suspicion and timely treatment is essential to prevent adverse outcomes.
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  • 文章类型: Case Reports
    噬血细胞淋巴组织细胞增多症(HLH)是一种罕见的,以抗原呈递细胞持续激活和多系统炎症为特征的危及生命的疾病。埃里希菌病是一种蜱虫感染,主要感染白细胞,并可出现各种症状,包括发烧,疲劳,和多系统并发症。这里,我们介绍了一名58岁女性因尿路感染住院。她的医院课程因HLH而变得复杂,后来被发现是由恰菲埃里希菌感染沉淀的。患者对多西环素治疗没有反应,出现多器官衰竭,并在几周的治疗试验后去世。
    Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening condition characterized by the persistent activation of antigen-presenting cells and multisystemic inflammation. Ehrlichiosis is a tick-born infection that primarily infects the white blood cells and can present with a variety of symptoms, including fever, fatigue, and multisystemic complications. Here, we present a 58-year-old female hospitalized for a urinary tract infection. Her hospital course was complicated by HLH, which was later discovered to be precipitated by an Ehrlichia chaffeensis infection. The patient did not respond to the doxycycline treatment, developed multiorgan failure, and passed away after a few weeks of treatment trials.
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  • 文章类型: Case Reports
    弥漫性大B细胞淋巴瘤(DLBCL)是非霍奇金淋巴瘤的最常见形式,很少表现为原发性胃肠道恶性肿瘤。原发性胃肠道淋巴瘤(PGIL)与穿孔和腹膜炎的显著风险相关,死亡率很高。在这里,我们描述了一名先前健康的22岁男性的新诊断的PGIL病例,该病例表现为新发作的腹痛伴腹泻。早期住院过程的特征是腹膜炎和严重的感染性休克。尽管有多种手术干预和复苏努力,患者的病情继续恶化,直到心脏骤停并在医院第5天死亡。尸检后病理诊断为回肠末端和盲肠的DLBCL。通过化疗方案的早期干预和恶性组织的手术切除可以改善这些患者的预后。该报告强调了DLBCL是胃肠道穿孔的罕见原因,可导致严重的多器官衰竭和死亡。
    Diffuse large B-cell lymphoma (DLBCL) is the most common form of non-Hodgkin\'s lymphoma and can rarely present as a primary gastrointestinal malignancy. Primary gastrointestinal lymphoma (PGIL) is associated with a significant risk of perforation and peritonitis, with high rates of mortality. Here we describe a case of a newly diagnosed PGIL in a previously healthy 22-year-old male presenting for new-onset abdominal pain with diarrhea. Early hospital course was characterized by peritonitis and severe septic shock. Despite multiple surgical interventions and resuscitative efforts, the patient\'s condition continued to deteriorate until cardiac arrest and death on hospital day five. A diagnosis of DLBCL of the terminal ileum and cecum was made by pathology post-mortem. The prognosis for these patients can be improved through early intervention with chemotherapy regimens and surgical resection of the malignant tissue. This report highlights DLBCL as a rare cause of gastrointestinal perforation that can culminate in precipitous multiorgan failure and death.
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  • 文章类型: Case Reports
    鼻窦炎和鼻中隔前蜂窝织炎是常见的急诊科(ED)条件,虽然罕见和致命的模仿可以以类似的方式存在。我们介绍了一例模仿鼻窦炎和鼻中隔前蜂窝织炎的自然杀伤(NK)/T细胞淋巴瘤。这种情况的诊断可能包括成像模式,如CT和MRI,虽然确诊需要组织活检.治疗干预包括化疗和放疗,对手术清创作用不大。治疗中可能发生并发症,包括噬血细胞淋巴组织细胞增多症。尽管有标准的治疗方法,面部淋巴瘤的死亡率仍然很高.
    Sinusitis and pre-septal cellulitis are common emergency department (ED) conditions, though rare and lethal mimics can present in a similar manner. We present a case of natural killer (NK)/T-cell lymphoma mimicking sinusitis and pre-septal cellulitis. Diagnosis of this condition may include imaging modalities such as CT and MRI, though definitive diagnosis requires tissue biopsy. Therapeutic interventions involve chemotherapy and radiation, with little role for surgical debridement. Complications in treatment can occur including hemophagocytic lymphohistiocytosis. Despite standard treatments, mortality remains high for cases of facial lymphoma.
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  • 文章类型: Case Reports
    Hemophagocytic lymphohistiocytosis (HLH) is an underdiagnosed, rare clinical syndrome, in particular secondary HLH, which mostly affects adults. HLH can be caused by malignancy, infections, autoimmune disorders, and, rarely, trauma. Here, we present the case of a patient who presented with anemia not responding to blood transfusion but improved after treatment with intravenous immunoglobulin. This case aims to highlight a rare presentation of this disease (HLH secondary to trauma) and to discuss the current HLH diagnostic criteria.
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