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BMC Cardiovascular Disorders Jul 2022Rumpel Leede sign (RLS) is a clinical presentation observed at the extremities due to pressure applied externally. The appearance ranges from scattered pin-point rashes...
BACKGROUND
Rumpel Leede sign (RLS) is a clinical presentation observed at the extremities due to pressure applied externally. The appearance ranges from scattered pin-point rashes to an entire arm covered with petechial hemorrhage depending upon the severity. This phenomenon is relatively uncommon in clinical practice.
CASE PRESENTATION
A 64 year old female patient developed a rash in the normal skin area below the compression area on the second day of single catheter coronary angiography. The patient's rash resolved without treatment after 3 days.
CONCLUSIONS
We report a case of hypertension and hyperlipidemia with a petechial rash on the skin under the tourniquet compressed by the radial artery after coronary angiography, which is consistent with the Rumpel-Leede phenomenon. clinicians should be watchful of these symptoms.
Topics: Coronary Angiography; Exanthema; Female; Humans; Middle Aged; Purpura; Radial Artery; Skin
PubMed: 35879660
DOI: 10.1186/s12872-022-02767-7 -
Journal of the European Academy of... Jan 2022
Topics: COVID-19; COVID-19 Vaccines; Humans; Purpura; SARS-CoV-2; Vaccination
PubMed: 34416052
DOI: 10.1111/jdv.17609 -
Blood Nov 2022
Topics: Humans; Purpura, Thrombocytopenic, Idiopathic; Platelet Count; Blood Platelets; Purpura, Thrombocytopenic
PubMed: 36355464
DOI: 10.1182/blood.2022017491 -
Stem Cell Research & Therapy Apr 2023Immune thrombocytopenia (ITP) is an acquired autoimmune disease involving a variety of immune cells and factors. Despite being a benign disease, it is still considered... (Review)
Review
Immune thrombocytopenia (ITP) is an acquired autoimmune disease involving a variety of immune cells and factors. Despite being a benign disease, it is still considered incurable due to its complex pathogenesis. Mesenchymal stem cells (MSCs), with low immunogenicity, pluripotent differentiation, and immunomodulatory ability, are widely used in a variety of autoimmune diseases. In recent years, impaired bone marrow mesenchymal stem cells (BMMSCs) were found to play an important role in the pathogenesis of ITP; and the therapeutic role of MSCs in ITP has also been supported by increasing evidence with encouraging efficacy. MSCs hold promise as a new approach to treat or even cure refractory ITP. Extracellular vesicles (EVs), as novel carriers in the "paracrine" mechanism of MSCs, are the focus of MSCs. Encouragingly, several studies suggested that EVs may perform similar functions as MSCs to treat ITP. This review summarized the role of MSCs in the pathophysiology and treatment of ITP.
Topics: Humans; Cell Differentiation; Extracellular Vesicles; Immunomodulation; Mesenchymal Stem Cells; Purpura, Thrombocytopenic, Idiopathic
PubMed: 37041587
DOI: 10.1186/s13287-023-03323-6 -
BioMed Research International 2020The novel coronavirus (SARS-CoV-2), the cause of coronavirus 2019 disease (COVID-19) pandemic, is associated with some cutaneous manifestations. Although the cutaneous... (Review)
Review
The novel coronavirus (SARS-CoV-2), the cause of coronavirus 2019 disease (COVID-19) pandemic, is associated with some cutaneous manifestations. Although the cutaneous presentations of COVID-19 are infrequent, it is of great importance for all clinicians to be aware of these manifestations, as it may contribute to sooner and better diagnosis and management of the disease, even in asymptomatic or paucisymptomatic patients. The reported cutaneous manifestations of COVID-19 are various, dispersed, and sometimes confusing. In this article, all reported cases to date were collected and classified under 6 major groups: maculopapular rash, urticaria, chilblain, vesicular lesions, livedo reticularis, and petechiae. Different characteristics of each group were discussed in detail as well.
Topics: Betacoronavirus; COVID-19; Chilblains; Coronavirus Infections; Exanthema; Female; Humans; Livedo Reticularis; Male; Pandemics; Pneumonia, Viral; Purpura; SARS-CoV-2; Skin Diseases, Viral; Urticaria
PubMed: 32724793
DOI: 10.1155/2020/1236520 -
Journal of Cutaneous Pathology May 2021Lichen aureus is a variant of pigmented purpuric dermatoses. The usual histopathology of lichen aureus is characterized by a subepidermal dense, band-like lymphocytic...
Lichen aureus is a variant of pigmented purpuric dermatoses. The usual histopathology of lichen aureus is characterized by a subepidermal dense, band-like lymphocytic infiltrate, extravasated erythrocytes, and hemosiderin deposits. We report three patients with lichen aureus on the extremities with similar clinical, dermoscopic, and histopathological findings characterized by a dense band-like relatively deep dermal infiltrate accompanied by extravasation of erythrocytes and hemosiderin deposits occasioning a resemblance to a lymphoproliferative disorder.
Topics: Adult; Dermoscopy; Diagnosis, Differential; Erythrocytes; Female; Hemosiderin; Humans; Immunohistochemistry; Lymphocytes; Lymphoproliferative Disorders; Male; Middle Aged; Pigmentation Disorders; Plasma Cells; Pseudolymphoma; Purpura; Skin Diseases
PubMed: 33368548
DOI: 10.1111/cup.13948 -
Frontiers in Immunology 2023Primary immune thrombocytopenia (ITP) is the most common acquired autoimmune bleeding disorder among children. While glucocorticoids are the primary first-line treatment...
OBJECTIVE
Primary immune thrombocytopenia (ITP) is the most common acquired autoimmune bleeding disorder among children. While glucocorticoids are the primary first-line treatment for ITP treatment, they prove ineffective in certain patients. The challenge of identifying biomarkers capable of early prediction regarding the response to glucocorticoid therapy in ITP persists. This study aimed to identify ideal biomarkers for predicting glucocorticoid efficacy in patients with ITP using plasma proteomics.
METHODS
A four-dimensional data-independent acquisition approach was performed to determine the differentially expressed proteins in plasma samples collected from glucocorticoid-sensitive (GCS) (n=18) and glucocorticoid-resistant (GCR) (n=17) children with ITP treated with prednisone. The significantly differentially expressed proteins were selected for enzyme-linked immunosorbent assay validation in a cohort conprising 65 samples(30 healthy controls, 18 GCS and 17 GCR children with ITP). Receiver operating characteristics curves, calibration curves, and clinical decision curve analysis were used to determine the diagnostic efficacy of this method.
RESULTS
47 differentially expressed proteins (36 up-regulated and 11 down-regulated) were identified in the GCR group compared with the GCS group. The significantly differentially expressed proteins myosin heavy chain 9 (MYH9) and fetuin B (FETUB) were selected for enzyme-linked immunosorbent assay validation. The validation results were consistent with the proteomics analyses. Compared with the GCS group, the GCR group exhibited a significantly reduced the plasma concentration of MYH9 and elevated the plasma concentration of FETUB. Furthermore, the receiver operating characteristics curves, calibration curves, and clinical decision curve analysis demonstrated good diagnostic efficacy of these validated biomarkers.
CONCLUSION
This study contributes to the establishment of objective biological indicators for precision therapy in children with ITP. More importantly, the proteins MYH9 and FETUB hold potential as a foundation for making informed decisions regarding alternative treatments for drugresistant patients, thereby preventing treatment delays.
Topics: Humans; Child; Glucocorticoids; Purpura, Thrombocytopenic, Idiopathic; Proteomics; Prednisone; Biomarkers
PubMed: 38162645
DOI: 10.3389/fimmu.2023.1301227 -
Journal of Thrombosis and Haemostasis :... Jan 2021Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare, life-threatening disease that causes systemic platelet-rich microthrombi with multiorgan damage.... (Review)
Review
Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare, life-threatening disease that causes systemic platelet-rich microthrombi with multiorgan damage. The historical treatment is based on therapeutic plasma exchange (TPE) and immunosuppression. Despite survival rates exceeding 85%, unfavorable outcomes including refractoriness, death, and exacerbations of the disease during treatment still calls for a better management strategy. Caplacizumab (Cablivi) appeared recently as a new treatment in iTTP. By inhibiting binding of von Willebrand factor to platelets, caplacizumab prevents platelets aggregation and the formation of microthrombi. Two pivotal randomized controlled trials have provided positive results where the use of caplacizumab is associated with faster platelet count recovery and less unfavorable outcomes. The other strength of this agent is an impressive alleviation in the burden of care, consisting in less TPE sessions and lower volumes of plasma to achieve remission, as well as substantial shortening in the length of hospitalization. However, since the recent approval of caplacizumab for the treatment of iTTP on the basis of these studies, debates remain regarding its systematic use in this indication. Should all patients be benefited from caplacizumab? Should we reserve caplacizumab only to the more severe patients? Should caplacizumab be initiated frontline or as a salvage therapy? If applicable, how should we select patients for caplacizumab? Last, is caplacizumab treatment cost-effective? This review aims at addressing these specific questions at a time when iTTP is entering the area of targeted therapies.
Topics: ADAMTS13 Protein; Humans; Plasma Exchange; Purpura, Thrombotic Thrombocytopenic; Single-Domain Antibodies
PubMed: 33236389
DOI: 10.1111/jth.15194 -
EMBO Molecular Medicine Oct 2015Mitochondrial disorders are a group of highly invalidating human conditions for which effective treatment is currently unavailable and characterized by faulty energy... (Review)
Review
Mitochondrial disorders are a group of highly invalidating human conditions for which effective treatment is currently unavailable and characterized by faulty energy supply due to defective oxidative phosphorylation (OXPHOS). Given the complexity of mitochondrial genetics and biochemistry, mitochondrial inherited diseases may present with a vast range of symptoms, organ involvement, severity, age of onset, and outcome. Despite the wide spectrum of clinical signs and biochemical underpinnings of this group of dis-orders, some common traits can be identified, based on both pathogenic mechanisms and potential therapeutic approaches. Here, we will review two peculiar mitochondrial disorders, ethylmalonic encephalopathy (EE) and mitochondrial neurogastrointestinal encephalomyopathy (MNGIE), caused by mutations in the ETHE1 and TYMP nuclear genes, respectively. ETHE1 encodes for a mitochondrial enzyme involved in sulfide detoxification and TYMP for a cytosolic enzyme involved in the thymidine/deoxyuridine catabolic pathway. We will discuss these two clinical entities as a paradigm of mitochondrial diseases caused by the accumulation of compounds normally present in traces, which exerts a toxic and inhibitory effect on the OXPHOS system.
Topics: Brain Diseases, Metabolic, Inborn; Humans; Intestinal Pseudo-Obstruction; Mitochondrial Diseases; Mitochondrial Encephalomyopathies; Mitochondrial Proteins; Muscular Dystrophy, Oculopharyngeal; Mutation; Nucleocytoplasmic Transport Proteins; Ophthalmoplegia; Purpura; Thymidine Phosphorylase
PubMed: 26194912
DOI: 10.15252/emmm.201505040 -
Haematologica Nov 2020
Topics: Child; Humans; Inosine Triphosphate; Purpura, Thrombocytopenic, Idiopathic; Registries; Splenectomy; Thrombocytopenia
PubMed: 33131241
DOI: 10.3324/haematol.2020.261099