eosinophilia

嗜酸性粒细胞增多症
  • 文章类型: Case Reports
    嗜酸性粒细胞增多是一种罕见的疾病,定义为绝对嗜酸性粒细胞计数持续升高大于1.5x109/L和/或组织嗜酸性粒细胞增多。这种情况可能是由许多不同的病因引起的,血液学(克隆)和非血液学(反应性)。反应性嗜酸性粒细胞增多症包括所有疾病,包括感染。嗜酸性粒细胞增多症患者可能由于多器官损伤而经历一系列临床后果,包括神经系统和血栓性并发症,与器官功能障碍和潜在危及生命的后遗症有关。脑静脉血栓形成(CVT)是用于描述脑中静脉和/或静脉窦的血栓性闭塞的术语。这种情况可能发生在所有年龄段,与嗜酸性粒细胞增多有关的CVT是一种罕见的疾病。由于血栓形成会导致脑引流阻塞,因此必须迅速进行疾病诊断。静脉充血,脑脊液重吸收中断,缺血性神经元损伤,脑水肿,出血,导致严重的神经系统并发症.由于嗜酸性粒细胞增多引起的CVT颅内出血的管理对于临床医生来说是一项具有挑战性的任务。基于抗凝治疗,全身性皮质类固醇,颅内压升高的管理,以及抗凝剂引起的潜在进行性出血。患者的预后通常取决于早期检测,提示,和适当的治疗。在这个案例报告中,我们讨论了一例罕见的儿童CVT伴嗜酸性粒细胞增多和登革热血清学阳性,在颅内出血的情况下,启发了在这个复杂场景的管理中考虑个性化策略的重要性。
    Hypereosinophilia is a rare condition, defined as a persistent elevation of absolute eosinophil count greater than 1.5x109/L and/or tissue eosinophilia. This condition can be caused by numerous different etiologies, both hematological (clonal) and non-hematological (reactive). Reactive hypereosinophilia encompasses all disorders, including infections. Patients with hypereosinophilia may experience a spectrum of clinical consequences due to multiple organ damage, including neurologic and thrombotic complications, associated with organ dysfunction and potentially life-threatening sequelae. Cerebral venous thrombosis (CVT) is the term used to describe thrombotic occlusion of veins and/or venous sinuses in the brain. This condition can occur at all ages and CVT related to hypereosinophilia is a rare disease. Diagnosis of the disease must be done quickly because thrombosis causes blockage of cerebral drainage, venous congestion, disruption of cerebrospinal fluid reabsorption, ischemic neuronal damage, cerebral edema, and hemorrhage, leading to severe neurological complications. Management of intracranial hemorrhage from CVT due to hypereosinophilia is a challenging task for clinicians, based on anticoagulation therapy, systemic corticosteroid, management of elevated intracranial pressure, and potentially progressive hemorrhage due to anticoagulant. The outcome of the patient generally relies on early detection, prompt, and appropriate treatment. In this case report, we discuss a rare case of CVT with hypereosinophilia and positive dengue serology in a child, in the context of intracranial hemorrhage, enlightening the importance of considering a personalized strategy in the management of this complex scenario.
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    文章类型: Journal Article
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  • 文章类型: Case Reports
    背景:嗜酸性实性和囊性肾细胞癌(ESC-RCC)是一种新型的肾细胞癌亚型,其特征是相对较低的发病率和惰性行为。我们报告了一例罕见的ESC-RCC并发单个肾脏中的透明细胞肾细胞癌(ccRCC)。
    方法:一名48岁男性,在体检中发现左肾有混合回声肿块。他没有血尿和侧腹疼痛的病史。腹部CT扫描显示3.0*1.9*2.5cm3肿块,左肾下极边界不清楚。腹部MRI显示左肾两个大小不同的结节,暗示了肿瘤的可能性.患者接受了肾次全切除术,术后病理结果提示ESC-RCC合并ccRCC。在12个月的随访中,患者恢复良好,没有肿瘤复发。
    结论:我们报道了一例肾脏复合肿瘤,包括罕见的ESC-RCC和更常见的ccRCC。影像学结合术后病理检查对于这些罕见肿瘤的明确诊断至关重要。
    BACKGROUND: Eosinophilic solid and cystic renal cell carcinoma (ESC-RCC) is a novel subtype of renal cell carcinoma characterized by its relatively low incidence and indolent behavior. We report a rare case of ESC-RCC concurrent with clear cell renal cell carcinoma (ccRCC) in a single kidney.
    METHODS: A 48-year-old male, was found to have a mixed echogenic mass in the left kidney during a physical examination. He has no history of hematuria and flank pain. An abdominal CT scan revealed a 3.0 * 1.9 * 2.5 cm3 mass with unclearly bordered at the lower pole of the left kidney. Abdominal MRI showed two nodules of different sizes in the left kidney, suggesting the possibility of a tumor. The patient underwent a subtotal nephrectomy, and the postoperative pathological results indicated ESC-RCC combined with ccRCC. The patient recovered well without tumor recurrence during the 12-month follow-up.
    CONCLUSIONS: We reported a case of renal composite tumors, comprising the rare ESC-RCC and the more common ccRCC. Imaging combined with postoperative pathological examination is crucial for the definitive diagnosis of these rare tumors.
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  • 文章类型: Case Reports
    嗜酸性筋膜炎(EF)由于其罕见且与硬皮病相似,仍然是诊断挑战。本病例报告旨在对EF的临床细微差别进行有凝聚力的探索,强调准确诊断和有效管理的重要性。一名52岁男性出现双侧前臂和小腿硬化,伴随着红斑,瘙痒,在我们诊所就诊前四个月疼痛。症状最初在前臂双侧出现,并发展到涉及小牛,远端臂,和大腿。临床检查显示前臂和小腿上有对称斑块,以红斑为特征,hyper,和向近侧延伸的低色素元素,积极的“凹槽征”和中度膝关节屈曲困难。尽管有这些发现,病人一般情况良好,无其他明显临床体征。最初的实验室发现显示嗜酸性粒细胞百分比水平略有增加,C反应蛋白(CRP)升高,正常红细胞沉降率(ESR),抗核和硬皮病特异性抗体阴性。磁共振成像(MRI)显示筋膜信号增强和增厚,而筋膜-肌肉活检显示明显的水肿和炎性淋巴浆细胞浸润,与EF诊断一致。患者对全身性皮质类固醇表现出良好的反应。EF主要影响30至60岁的男性,其特征是突然发作且病因不明确。鉴别诊断需要仔细排除硬皮病和其他模拟条件。皮肤肌肉活检和MRI等诊断方式可显示出炎性浸润和筋膜增厚等特征性发现。准确诊断和鉴别硬皮病至关重要,包括糖皮质激素和免疫抑制剂的早期干预可改善长期结局。
    Eosinophilic fasciitis (EF) remains a diagnostic challenge due to its rarity and resemblance to scleroderma. This case report aims to provide a cohesive exploration of EF\'s clinical nuances, emphasizing the importance of accurate diagnosis and effective management. A 52-year-old male developed bilateral forearm and calf hardening, along with erythema, pruritus, and pain four months prior to the presentation in our Clinic. The symptoms initially debuted bilaterally in the forearms and progressed to involve the calves, distal arms, and thighs. Clinical examination revealed symmetrical plaques on forearms and calves, featuring erythematous, hyper, and hypopigmented elements extending proximally, a positive \"groove sign\" and a moderate difficulty in knee joint flexion. Despite these findings, the patient was generally in good condition, without any other notable clinical signs. Initial laboratory findings showed slightly increased percentual eosinophil levels, elevated C-reactive protein (CRP), normal erythrocyte sedimentation rate (ESR), and negative antinuclear and scleroderma specific antibodies. Magnetic resonance imaging (MRI) demonstrated enhanced fascial signal and thickening while the fascia-muscle biopsy revealed marked edema and inflammatory lymphoplasmacytic infiltrate, consistent with the diagnosis of EF. The patient showed a favorable response to systemic corticosteroids. EF predominantly affects males aged 30 to 60 and is characterized by a sudden onset and unclear etiological factors. Differential diagnosis requires careful exclusion of scleroderma and other mimicking conditions. Diagnostic modalities such as skin-muscle biopsy and MRI reveal characteristic findings like inflammatory infiltrate and fascial thickening. Accurate diagnosis and differentiation from scleroderma are crucial, with early intervention involving glucocorticoids and immunosuppressive agents improving long-term outcomes.
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  • 文章类型: Case Reports
    嗜酸性粒细胞增多综合征是一种罕见的以嗜酸性粒细胞介导的器官损伤为特征的全身性疾病。心脏受累是常见的,通常发生在连续阶段。我们提供了两个病例,证明了嗜酸性粒细胞增多介导的心肌病的这些不同阶段和表现,多模态成像对诊断至关重要。更重要的是,他们证明,第一次,嗜酸性粒细胞计数与患者临床演变之间的分离,提示即使在嗜酸性粒细胞增多得到控制后仍需要密切随访.
    嗜酸性粒细胞增多综合征的心脏受累通常发生在三个阶段-坏死,血栓形成,和纤维化。虽然心脏损伤是由嗜酸性粒细胞介导的,血液嗜酸性粒细胞计数和患者的临床演变是分离的。因此,嗜酸性粒细胞计数本身并不是临床进化的适当标志,即使在嗜酸性粒细胞增多得到控制后,也应继续心脏随访。
    Hypereosinophilic syndrome is a rare systemic condition characterized by eosinophil-mediated organ damage. Cardiac involvement is common and typically occurs in sequential stages. We present two cases that demonstrate these different stages and presentations of eosinophilia-mediated myocardial disease, where multimodality imaging was essential for the diagnosis. More importantly, they demonstrate, for the first time, the dissociation between the eosinophil count and patients\' clinical evolution, suggesting the need for close follow up even after the eosinophilia has been controlled.
    UNASSIGNED: Cardiac involvement in hypereosinophilic syndrome typically occurs in three stages - necrotic, thrombotic, and fibrotic. Although cardiac damage is mediated by eosinophils, the blood eosinophil count and patients\' clinical evolution are dissociated. Therefore, eosinophil count on its own is not an adequate marker of clinical evolution, and cardiac follow up should be continued even after the eosinophilia has been controlled.
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  • 文章类型: Case Reports
    背景:非典型慢性粒细胞白血病(aCML)是一种高度侵袭性类型的血癌,属于骨髓增生异常/骨髓增殖性肿瘤(MDS/MPN)。在世界卫生组织第五版的肿瘤分类中,该类别已更名为中性粒细胞增多症的MDS/MPN。尽管嗜酸性粒细胞增多通常在血癌中观察到,在aCML中很少见。
    方法:本研究提供一例aCML病例,该病例在患者出现嗜酸性粒细胞增多6年后确诊。病人接受了检查以排除其他原发性和继发性疾病,但嗜酸性粒细胞增多仍无法解释.用皮质类固醇和羟基脲治疗已证明无效。六年后,病人出现了白细胞的增加,主要是中性粒细胞。排除了其他可能的诊断后,形态学和分子遗传学的结合发现导致了aCML的诊断.患者对阿扎胞苷治疗反应良好。
    结论:本研究总结了aCML的诊断和治疗现状,并讨论了嗜酸性粒细胞增多和aCML之间的可能联系。
    BACKGROUND: Atypical chronic myeloid leukemia (aCML) is a highly aggressive type of blood cancer that falls under the category of myelodysplastic/myeloproliferative neoplasms (MDS/MPN). In the fifth edition of the WHO classification of tumors, this category has been renamed MDS/MPN with neutrophilia. Although eosinophilia is commonly observed in blood cancers, it is rarely seen in aCML.
    METHODS: This study presents a case of aCML that was diagnosed six years after the patient developed eosinophilia. The patient had undergone tests to rule out other primary and secondary diseases, but the eosinophilia remained unexplained. Treatment with corticosteroids and hydroxyurea had proven ineffective. Six years later, the patient experienced an increase in white blood cells, primarily neutrophils. After ruling out other possible diagnoses, a combination of morphologic and molecular genetic findings led to the diagnosis of aCML. The patient responded well to treatment with azacitidine.
    CONCLUSIONS: This study summarizes the current state of aCML diagnosis and management and discusses the possible connection between eosinophilia and aCML.
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  • 文章类型: Case Reports
    获得性反应性穿孔胶原病(ARPC)是一种罕见的皮肤病学病症,由改变的胶原纤维通过表皮穿孔定义。糖尿病或肾脏疾病等潜在疾病的存在有助于ARPC诊断。尽管已经报道了与ARPC有关的皮疹,确切的致病因素和机制尚不清楚.这里,我们介绍了一例由创伤引发的ARPC的独特病例,该病例发生在一名67岁男性中,但没有并发全身改变.ARPC伴嗜酸性粒细胞增多的诊断是在综合诊断测试后做出的。包括临床表现,组织学结果,还有血液测试,排除其他可能的疾病。有趣的是,组织病理学检查显示胶原蛋白在不同的组织切片处渗透到表皮中。此外,我们回顾了有关ARPC的现有文献,记录了因果关系。为了帮助确认诊断,临床医生必须注意ARPC的创伤性诱因及其罕见的嗜酸性粒细胞增多表现。
    Acquired reactive perforating collagenosis (ARPC) is a rare dermatological disorder condition defined by the perforation of altered collagen fibers through the epidermis. The presence of underlying conditions such as diabetes or renal disease is helpful in the ARPC diagnosis. Although skin rashes related to ARPC have been reported, the exact causative factors and mechanisms remain unclear. Here, we present a unique case of ARPC triggered by trauma in a 67-year-old male without concurrent systemic alterations. The diagnosis of ARPC with eosinophilia was made following comprehensive diagnostic testing, including clinical presentation, histological results, and blood tests, ruling out other possible diseases. Intriguingly, the histopathological examination revealed collagen penetration into the epidermis at different tissue sections. In addition, we reviewed existing literature on ARPC, which documented the causation. To help confirm the diagnosis, clinicians have to pay attention to traumatic triggers for ARPC and its rare manifestation with eosinophilia.
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  • 文章类型: Case Reports
    由于颌面部间隙感染,一名79岁的男子接受了手术引流和2周的头孢菌素治疗(双侧颌下,submentum,和左脸)。然而,他经历了厌食症,恶心,呕吐,在接下来的两个月里消瘦。最初认为可能存在恶性肿瘤,因此进行了一系列检查。实验室调查显示炎症标志物增加和显著的嗜酸性粒细胞增多,这似乎是一种血液系统疾病。结合胃肠内镜和组织学检查,患者被诊断为嗜酸性粒细胞性胃肠炎(EGE).停止抗生素治疗和皮质类固醇给药后,我们的病人经历了他的临床病情的快速进展。尽管发病率低,胃肠道疾病原因不明的患者应考虑EGE,嗜酸性粒细胞升高,等等。
    A 79-year-old man underwent operative drainage and 2-week cephalosporin treatment due to a maxillofacial space infection (bilateral submaxillaris, submentum, and left face). However, he experienced anorexia, nausea, vomiting, and emaciation in the following 2 months. It was initially considered that a malignancy might be present, thus a series of examinations were performed. Laboratory investigations showed increases in inflammatory markers and a significant eosinophilia, which seemed to be a hematological system disease. Combined with the gastrointestinal endoscopes and histology examination, the patient was diagnosed with eosinophilic gastroenteritis (EGE). After cessation of antibiotic treatment and administration of corticosteroid, our patient experienced a rapid progress in his clinical condition. Despite the low incidence, EGE should be considered in patients with unknown cause of gastrointestinal disorder, elevated eosinophilia, and so on.
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  • 文章类型: Case Reports
    血管免疫母细胞性T细胞淋巴瘤(AITL)是一种罕见的非霍奇金淋巴瘤(NHL)。我们介绍了一例60岁的女性,她因疲劳而去急诊科(ED)就诊,反复发烧,减肥,和腺病六个月。实验室检查结果显示贫血,淋巴细胞增多,嗜酸性粒细胞增多,血小板增多症,胆汁淤积,低蛋白血症,和低蛋白血症.腹盆腔计算机断层扫描(CT)显示多发腺病。在门诊进行的淋巴结活检结果尚无定论。稍后,入院期间,患者接受了正电子发射断层扫描-计算机断层扫描(PET-CT),显示一个整体切除的宫颈腺病簇。组织学证实了AITL的诊断。医疗团队开始化疗,但由于疾病进展而选择了独家对症治疗。患者在诊断后六个月死亡。AITL的波动和非特异性表现可能会阻碍和延迟明确的诊断,因此影响治疗和预后。
    Angioimmunoblastic T-cell lymphoma (AITL) is a rare type of non-Hodgkin lymphoma (NHL). We present a case of a 60-year-old female who attended the emergency department (ED) with fatigue, recurrent fever, weight loss, and adenopathy for six months. Laboratory findings showed anemia, lymphocytosis, eosinophilia, thrombocytosis, cholestasis, hypoproteinemia, and hypoalbuminemia. Abdominopelvic computed tomography (CT) revealed multiple adenopathies. A lymph node biopsy yielded inconclusive results in the outpatient clinic. Later, during admission, the patient underwent a positron emission tomography-computed tomography (PET-CT), revealing a cervical adenopathy cluster that was excised en bloc. Histology confirmed the diagnosis of AITL. The medical team initiated chemotherapy but opted for exclusive symptomatic treatment due to disease progression. The patient died six months after diagnosis. The fluctuating and nonspecific presentation of AITL can hinder and delay definitive diagnosis, therefore impacting treatment and prognosis.
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  • 文章类型: Case Reports
    嗜酸性粒细胞增多在所有年龄段都是罕见的,尤其是严重的时候,持久性,和进步。我们描述了足月沙特女性新生儿严重嗜酸性粒细胞增多的临床特征和病程。发热性呼吸系统疾病随着外周血白细胞和嗜酸性粒细胞计数的逐渐增加而发展。达到44.9%的白细胞和57,000个细胞/μl的绝对值。不同的病因检查(对于病毒,细菌,免疫缺陷,高IgE综合征,基因突变)揭示了极高的CMV抗原血症和STAT1基因的纯合突变。氧气和抗病毒治疗缓解了焦虑。类固醇在24小时内对外周血计数产生了巨大的反应。在家中进行为期6周的抗病毒和类固醇治疗后,她的一般情况很好。结论:虽然病理罕见,当存在对病毒感染的非典型免疫反应时,考虑遗传性疾病是很重要的。
    Hypereosinophilia is a rare presentation in all age groups, particularly when it is severe, persistent, and progressive. We describe the clinical characteristics and course of severe hypereosinophilia in a full-term Saudi female neonate. A febrile respiratory illness evolved with a progressive increase in peripheral blood leukocyte and eosinophil counts, reaching 44.9% of leukocytes and an absolute value of 57,000 cells/µl. Different etiological examinations (for viral, bacterial, immunodeficiency, hyper IgE syndrome, gene mutations) revealed extremely high CMV antigenemia and a homozygous mutation in the STAT1 gene. Anhelation was relieved by oxygen and anti-viral treatment. Steroids brought a dramatic response in peripheral blood counts within 24 h. After a 6-week course of antiviral and steroid treatment at home, she had an excellent general condition. Conclusion: Although a rare pathology, it is important to consider genetic disorders when there is an atypical immune response to viral infections.
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