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Sultan Qaboos University Medical Journal Nov 2022
Topics: Humans; Purpura; Pigmentation Disorders; Exanthema; Eczema
PubMed: 36407701
DOI: 10.18295/squmj.1.2022.004 -
Blood Jan 2023
Topics: Pregnancy; Female; Humans; Purpura, Thrombocytopenic, Idiopathic; Cohort Studies; Thrombocytopenia; Patients
PubMed: 36602822
DOI: 10.1182/blood.2022018082 -
Clinical and Experimental Dermatology Apr 2021The current COVID-19 pandemic is caused by the SARS-CoV-2 coronavirus. The initial recognized symptoms were respiratory, sometimes culminating in severe respiratory... (Review)
Review
The current COVID-19 pandemic is caused by the SARS-CoV-2 coronavirus. The initial recognized symptoms were respiratory, sometimes culminating in severe respiratory distress requiring ventilation, and causing death in a percentage of those infected. As time has passed, other symptoms have been recognized. The initial reports of cutaneous manifestations were from Italian dermatologists, probably because Italy was the first European country to be heavily affected by the pandemic. The overall clinical presentation, course and outcome of SARS-CoV-2 infection in children differ from those in adults as do the cutaneous manifestations of childhood. In this review, we summarize the current knowledge on the cutaneous manifestations of COVID-19 in children after thorough and critical review of articles published in the literature and from the personal experience of a large panel of paediatric dermatologists in Europe. In Part 1, we discuss one of the first and most widespread cutaneous manifestations of COVID-19, chilblain-like lesions, and in Part 2 we expanded to other manifestations, including erythema multiforme, urticaria and Kawasaki disease-like inflammatory multisystemic syndrome. In this part of the review, we discuss the histological findings of COVID-19 manifestations, and the testing and management of infected children for both COVID-19 and any other pre-existing conditions.
Topics: Adolescent; Antibodies, Monoclonal, Humanized; COVID-19; COVID-19 Testing; Child; Dermatologic Agents; Exanthema; Humans; Nicolau Syndrome; Pityriasis Rosea; Purpura; SARS-CoV-2; Skin Diseases, Viral; Urticaria
PubMed: 33207021
DOI: 10.1111/ced.14483 -
Medicina Clinica Nov 2022
Topics: Humans; COVID-19; Purpura; Cryoglobulinemia
PubMed: 35995607
DOI: 10.1016/j.medcli.2022.06.011 -
Journal of Thrombosis and Haemostasis :... Jun 2023Severe deficiency in ADAMTS-13 (<10%) and the loss of von Willebrand factor-cleaving function can precipitate microvascular thrombosis associated with thrombotic...
BACKGROUND
Severe deficiency in ADAMTS-13 (<10%) and the loss of von Willebrand factor-cleaving function can precipitate microvascular thrombosis associated with thrombotic thrombocytopenic purpura (TTP). Patients with immune-mediated TTP (iTTP) have anti-ADAMTS-13 immunoglobulin G antibodies that inhibit ADAMTS-13 function and/or increase ADAMTS-13 clearance. Patients with iTTP are treated primarily by plasma exchange (PEX), often in combination with adjunct therapies that target either the von Willebrand factor-dependent microvascular thrombotic processes (caplacizumab) or the autoimmune components (steroids or rituximab) of the disease.
OBJECTIVES
To investigate the contributions of autoantibody-mediated ADAMTS-13 clearance and inhibition in patients with iTTP at presentation and through the course of the PEX therapy.
PATIENTS/METHODS
Anti-ADAMTS-13 immunoglobulin G antibodies, ADAMTS-13 antigen, and activity were measured before and after each PEX in 17 patients with iTTP and 20 acute TTP episodes.
RESULTS
At presentation, 14 out of 15 patients with iTTP had ADAMTS-13 antigen levels of <10%, suggesting a major contribution of ADAMTS-13 clearance to the deficiency state. After the first PEX, both ADAMTS-13 antigen and activity levels increased similarly, and the anti-ADAMTS-13 autoantibody titer decreased in all patients, revealing ADAMTS-13 inhibition to be a modest modifier of the ADAMTS-13 function in iTTP. Analysis of ADAMTS-13 antigen levels between consecutive PEX treatments revealed that the rate of ADAMTS-13 clearance in 9 out of 14 patients analyzed was 4- to 10-fold faster than the estimated normal rate of clearance.
CONCLUSION
These data reveal, both at presentation and during PEX treatment, that antibody-mediated clearance of ADAMTS-13 is the major pathogenic mechanism that causes ADAMTS-13 deficiency in iTTP. Understanding the kinetics of ADAMTS-13 clearance in iTTP may now enable further optimization of treatment of patients with iTTP.
Topics: Humans; Autoantibodies; Purpura, Thrombotic Thrombocytopenic; von Willebrand Factor; Purpura, Thrombocytopenic, Idiopathic; Thrombosis; ADAMTS13 Protein; Immunoglobulin G
PubMed: 36813118
DOI: 10.1016/j.jtha.2023.02.011 -
Anais Brasileiros de Dermatologia Jun 2018Purpura is defined as a visible hemorrhage in the skin or mucosa, which is not evanescent upon pressure. Proper classification allows a better patient approach due to... (Review)
Review
Purpura is defined as a visible hemorrhage in the skin or mucosa, which is not evanescent upon pressure. Proper classification allows a better patient approach due to its multiple diagnoses. Purpuras can be categorized by size, morphology, and other characteristics. The course varies according to the etiology, as do the diagnostic approach and treatment. This review discusses pigmented purpuras and some cutaneous vascular occlusion syndromes.
Topics: Antiphospholipid Syndrome; Calciphylaxis; Diagnosis, Differential; Humans; Pigmentation Disorders; Purpura; Purpura Fulminans; Skin; Skin Diseases, Vascular; Syndrome
PubMed: 29924250
DOI: 10.1590/abd1806-4841.20187459 -
Innere Medizin (Heidelberg, Germany) Dec 2022Thrombotic thrombocytopenic purpura (TTP) remains a serious illness with potentially life-threatening complications. The following case of a TTP patient describes...
Thrombotic thrombocytopenic purpura (TTP) remains a serious illness with potentially life-threatening complications. The following case of a TTP patient describes a serious relapse with exacerbation in spite of adequately initiated therapy and highlights the necessity of interdisciplinary expertise in the treatment of the disease.
Topics: Humans; Purpura, Thrombotic Thrombocytopenic; Recurrence
PubMed: 36194294
DOI: 10.1007/s00108-022-01408-7 -
Archives of Pathology & Laboratory... Aug 2023Immune thrombotic thrombocytopenic purpura (iTTP) is a rare but potentially fatal blood disorder resulting from acquired deficiency of plasma ADAMTS13, a metalloprotease... (Review)
Review
CONTEXT.—
Immune thrombotic thrombocytopenic purpura (iTTP) is a rare but potentially fatal blood disorder resulting from acquired deficiency of plasma ADAMTS13, a metalloprotease that cleaves endothelium-derived ultralarge von Willebrand factor. Standard of care for iTTP including therapeutic plasma exchange, caplacizumab, and immunosuppressives, known as triple therapy, has led to a significant reduction in the disease-related mortality rate. The first International Society of Thrombosis and Haemostasis TTP guideline stresses the importance of having plasma ADAMTS13 activity testing in the algorithm for diagnosis and management of iTTP. However, the predictive role of assessing plasma ADAMTS13 activity and inhibitors or other ADAMTS13-related parameters in patients with acute iTTP and during remission has not been systematically evaluated.
OBJECTIVE.—
To review and assess the predictive values of testing plasma ADAMTS13 activity, antigen, and inhibitors or anti-ADAMTS13 immunoglobulin G at various stages of disease in outcomes of iTTP.
DATA SOURCES.—
Peer-reviewed publications and personal experience.
CONCLUSIONS.—
We conclude that assessing ADAMTS13 biomarkers is not only essential for establishing the initial diagnosis, but also crucial for risk stratification and the early detection of disease recurrence. This may guide therapeutic interventions during acute episodes and for long-term follow-up of iTTP patients.
Topics: Humans; ADAMTS13 Protein; Biomarkers; Immunosuppressive Agents; Purpura, Thrombotic Thrombocytopenic; Thrombosis; von Willebrand Factor
PubMed: 36223210
DOI: 10.5858/arpa.2022-0050-RA -
British Journal of Haematology Apr 2017The management of patients with immune thrombocytopenia (ITP) is rapidly evolving. Over the last 15 years, a number of novel treatments have improved practice, with... (Review)
Review
The management of patients with immune thrombocytopenia (ITP) is rapidly evolving. Over the last 15 years, a number of novel treatments have improved practice, with many steroid-sparing agents and a reduction in the progression to splenectomy. Although this has improved clinical care, many therapeutic challenges remain. There is no diagnostic test, no biomarkers to direct treatment and few comparative studies to help management decisions. Development of up to date guidelines is difficult with little high-grade evidence. First line treatment continues to be steroids and intravenous immunoglobulins (IVIG) although both are often poorly tolerated and not curative. Common second line treatments include rituximab, immunosuppressive agents, such as azathioprine and mycophenolate mofetil, and the thrombopoietin receptor agonists romiplostim and eltrombopag. There are no comparative studies to decide between these agents and treatment is generally individualized, depending on comorbidity. Use of splenectomy has declined and is generally reserved for patients with chronic disease, although the exact position of splenectomy is subject to debate. Further understanding of the cause of disease in individual patients may help guide treatment. Randomized controlled studies of common treatments and novel treatments for refractory patients are urgently needed.
Topics: Clinical Decision-Making; Decision Trees; Disease Management; Humans; Practice Guidelines as Topic; Purpura, Thrombocytopenic, Idiopathic
PubMed: 28295192
DOI: 10.1111/bjh.14515 -
British Journal of Haematology Apr 2022The 100th anniversary of the first description of Thrombotic Thrombocytopenic Purpura (TTP) as a disease by Dr. Eli Moschcowitz approaches. For many decades, TTP... (Review)
Review
The 100th anniversary of the first description of Thrombotic Thrombocytopenic Purpura (TTP) as a disease by Dr. Eli Moschcowitz approaches. For many decades, TTP remained mostly a mysterious fatal condition, where diagnosis was often post-mortem. Initially a pentad of symptoms was identified, a pattern that later revealed to be fallible. Sporadic observations led to empiric interventions that allowed for the first impactful breakthrough in TTP treatment, almost 70 years after its first description: the introduction of plasma exchange and infusions as treatments. The main body of knowledge within the field was gathered in the latest three decades: patient registries were set and proved crucial for advancements; the general mechanisms of disease have been described; the diagnosis was refined; new treatments and biomarkers with improvements on prognosis and management were introduced. Further changes and improvements are expected in the upcoming decades. In this review, we provide a brief historic overview of TTP, as an illustrative example of the success of translational medicine enabling to rapidly shift from a management largely based on empiricism to targeted therapies and personalized medicine, for the benefit of patients. Current management options and present and future perspectives in this still evolving field are summarized.
Topics: ADAMTS13 Protein; Empiricism; Humans; Molecular Targeted Therapy; Plasma Exchange; Purpura, Thrombotic Thrombocytopenic
PubMed: 35146746
DOI: 10.1111/bjh.18040