[POEMS综合症:两例病例的报告]
Evelyn Ortiz1, José Lillo1, Gery Lara2
1Hospital Carlos Van Buren, Valparaíso, Chile.
概括
POEMS综合征是一种罕见的血细胞瘤,呈现出各种症状. 这项研究详细介绍了两起智利病例,突出了诊断标准和成功的梅尔法兰/甲治疗为稳定的患者结果.
科学领域:
- 血液学 血液学 血液学
- 神经学 神经学
- 在瘤学瘤学.
背景情况:
- POEMS综合征是一种罕见的多系统性疾病,与血细胞瘤有关.
- 病理生理学涉及益炎性细胞因子和血管内皮生长因子.
- 诊断依赖于国际骨髓瘤工作组的标准,尽管异常呈现也会发生.
研究的目的:
- 在卡洛斯·范·伯伦医院诊断和治疗的两例POEMS综合征.
- 为了在诊断标准中说明可变的临床表现.
- 报告使用特定治疗方案的治疗结果.
主要方法:
- 两名患者的回顾性病例系列分析.
- 国际骨髓瘤工作组对POEMS综合征的诊断标准的应用.
- 用梅尔法兰/德克萨米他松治疗方案的治疗.
主要成果:
- 两位患者都符合POEMS综合征的诊断标准,但表现出不同的小标准表现.
- 观察到多发性神经病变和未确定意义的单克隆性胃病变 (GMSI).
- 两名患者在治疗后的门诊监测下实现了稳定的疾病.
结论:
- POEMS综合征需要仔细诊断,即使具有非典型特征.
- 梅尔法兰/甲治疗方案在治疗POEMS综合征方面显得有效.
- 对患有POEMS综合征的患者来说,持续监测至关重要.
相关概念视频
Pulmonary Embolism I: Introduction
1
Pulmonary embolism (PE) occurs when a thrombus, fat or air embolus, amniotic fluid, or tumor tissue blocks one or more pulmonary arteries. These blockages originate in the venous system or the right side of the heart.EtiologyPE primarily arises from deep vein thrombosis (DVT) and other hypercoagulable states, such as inherited thrombophilias. Additional etiological factors include venous stasis, commonly seen in obesity, and endothelial injury from surgery and trauma. Less common causes include...
1
Acute Coronary Syndrome II: Pathophysiology and clinical manifestations
3
The pathophysiology of Acute Coronary Syndrome [ACD] involves several key processes:The main underlying cause of ACD is atherosclerosis, a chronic inflammatory disease characterized by the buildup of lipid-laden plaques within the coronary arteries.As the atherosclerotic plaque grows in the coronary artery, it may become unstable due to the formation of a lipid-rich core and a thin fibrous cap. Inflammatory cells within the plaque, such as macrophages, secrete enzymes that degrade the...
3
Myocarditis II: Clinical features and Diagnostic Tests
2
Myocarditis is an inflammation of the heart muscle. The symptoms vary widely, encompassing asymptomatic presentations to severe, acute manifestations.Clinical PresentationAsymptomatic cases: In some instances, myocarditis may be asymptomatic, with the infection resolving without intervention. These cases often go undetected unless discovered incidentally through diagnostic imaging or tests conducted for other reasons.General Early Symptoms: Early symptoms of myocarditis are non-specific and can...
2
Pulmonary Embolism II: Diagnostic Studies and Interprofessional Care
Diagnosing Pulmonary EmbolismDiagnosing pulmonary embolism (PE) involves clinical assessment and advanced imaging tests. The preferred diagnostic tool is the spiral (helical) CT scan or CT angiography (CTA), which uses intravenous contrast media to visualize the pulmonary vasculature and identify emboli.A ventilation-perfusion (V/Q) scan is an alternative for patients unable to receive contrast media. This scan includes both perfusion and ventilation scanning. Perfusion scanning involves...
Endocarditis II: Clinical features and Diagnostic Tests
2
Endocarditis can present various clinical features depending on the causative organism and the patient's underlying health conditions. Initially, the clinical features of infective endocarditis develop gradually, presenting with nonspecific symptoms that can be easily mistaken for other illnesses.General SymptomsEarly symptoms of infective endocarditis are fever, chills, weakness, malaise, fatigue, and weight loss. These symptoms reflect the systemic nature of the infection and the body's...
2
Acute Coronary SyndromeI: Introduction
3
Acute Coronary Syndrome (ACS) encompasses a spectrum of heart conditions caused by sudden obstruction of coronary arteries, typically resulting from the rupture of an atherosclerotic plaque and subsequent thrombus (blood clot) formation. This obstruction can lead to partial or complete blockage of blood flow, causing varying degrees of myocardial ischemia or infarction.ACS includes the following clinical entities:Unstable Angina (UA)Non-ST-Elevation Myocardial Infarction (NSTEMI)ST-Elevation...
3


