Related Experiment Video
Updated: Sep 20, 2025
10:08
Tachycardia-Induced Cardiomyopathy As a Chronic Heart Failure Model in Swine
Published on: February 17, 2018
13.6K
TEMPI Syndrome: Update on Clinical Features, Management, and Pathogenesis
Jian Xu1, Wenqi Liu1, Fengjuan Fan1
1Institute of Hematology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Frontiers in Endocrinology
|June 6, 2022
Summary
TEMPI syndrome, a rare multisystemic disease, presents with diverse symptoms, often leading to misdiagnosis. This review details its clinical features, pathophysiology, and treatment options for better understanding and management.
Area of Science:
- Hematology
- Nephrology
- Pulmonology
Background:
- TEMPI (telangiectasias, elevated erythropoietin level and erythrocytosis, monoclonal gammopathy, perinephric fluid collections, and intrapulmonary shunting) syndrome is a rare multisystemic disease.
- It is classified under "monoclonal gammopathy of clinical significance" but its etiology and pathogenesis remain largely unknown due to its rarity.
- Diverse and often hidden clinical manifestations contribute to frequent missed diagnoses and misdiagnoses.
Purpose of the Study:
- To review and summarize the reported clinical characteristics of TEMPI syndrome.
- To discuss current and potential therapeutic strategies for TEMPI syndrome, including relapsed/refractory cases.
- To provide an overview of the current understanding of TEMPI syndrome's pathophysiology.
Main Methods:
- Literature review of reported cases and studies on TEMPI syndrome.
- Synthesis of clinical data, including typical and atypical manifestations.
- Analysis of existing research on pathogenesis and treatment approaches.
Main Results:
- TEMPI syndrome exhibits a wide range of clinical features beyond the typical pentad.
- Recent findings include identification of more patients and reports of special clinical manifestations.
- Studies are emerging to elucidate the underlying pathogenesis of the syndrome.
Conclusions:
- Increased recognition of TEMPI syndrome is leading to a better understanding of its diverse clinical spectrum.
- Further research into pathogenesis is crucial for developing targeted therapies.
- Comprehensive management strategies are needed for patients with TEMPI syndrome, addressing both typical and atypical presentations.
Keywords:
TEMPI syndromeclinical manifestationsmonoclonal gammopathy of clinical significancepathogenesisplasma cell-directed treatmentMore Related Videos
Related Concept Videos
01:19Acute Coronary Syndrome II: Pathophysiology and Clinical Manifestations
55
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...
55
01:17Pericarditis III: Medical Management
33
The primary objectives of managing pericarditis are to determine the underlying cause, provide effective therapy for treatment and symptom relief, and promptly detect signs and symptoms of cardiac tamponade. The following outlines the essential aspects of medical management for pericarditis:ObjectivesDetermine the Cause: Identifying the underlying cause of pericarditis is crucial for targeted treatment. Causes include viral infections, autoimmune diseases, post-cardiac injury syndrome, and...
33
01:30Acute Pancreatitis II: Clinical Manifestations and Management
186
Acute pancreatitis presents a complex medical emergency characterized by rapid onset inflammation of the pancreas, demanding timely diagnosis and management to prevent complications. The condition primarily manifests through severe upper abdominal pain that often radiates to the back. This pain intensifies following the consumption of fatty foods. Accompanying symptoms such as nausea, vomiting, abdominal distention, fever, dyspnea, cyanosis, and jaundice can vary in intensity but significantly...
186
01:14Myocarditis III: Medical Management
22
Myocarditis: Comprehensive Medical ManagementMyocarditis, the heart muscle inflammation, requires a comprehensive medical management strategy that addresses the underlying cause, provides supportive care, manages symptoms, and reduces cardiac workload.Infections and Autoimmune CausesAdminister appropriate antimicrobial therapy when an infectious agent causes myocarditis. For instance, penicillin treats infections caused by Group A Streptococcus. In cases where autoimmune processes are...
22
01:27Myocarditis II: Clinical Features and Diagnostic Tests
34
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...
34
01:22Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies
73
The key clinical manifestations of Rheumatic heart disease (RHD) include several distinct cardiac symptoms.Carditis, a hallmark of acute rheumatic fever, involves inflammation of the heart's endocardium, myocardium, and pericardium. Chronic RHD often results from recurrent episodes of carditis. Its symptoms include the following:Murmurs are caused by valvular damage, especially to the mitral and aortic valves. Mitral stenosis or regurgitation is common, with characteristic heart murmurs...
73