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Acta Medica Indonesiana Apr 2021Thrombotic thrombocytopenic purpura is a rare but life threatening medical condition. Early recognition and treatment of thrombotic thrombocytopenic purpura is important...
Thrombotic thrombocytopenic purpura is a rare but life threatening medical condition. Early recognition and treatment of thrombotic thrombocytopenic purpura is important especially in patients who do not present with the classic pentad to reduce the high mortality. Herein, we describe a case of a patient who does not fulfil the classic pentad features thrombotic thrombocytopenic purpura that was induced by dengue fever. The patients' initial full blood picture did not have all the typical features of microangiopathic haemolytic anaemia but there were fragmented red blood cells. However, even a small number of fragmented red blood cells in the peripheral blood should alert physicians of the possible diagnosis of thrombotic thrombocytopenic purpura together with other symptoms. Furthermore, signs and symptoms of thrombotic thrombocytopenic purpura and dengue fever can overlap such as fever, thrombocytopenia, neurological deficit mimicking dengue encephalopathy and dengue induced acute kidney injury.
Topics: Adult; Dengue; Humans; Male; Purpura, Thrombotic Thrombocytopenic
PubMed: 34251350
DOI: No ID Found -
Blood Apr 2022
Topics: ADAMTS13 Protein; COVID-19; COVID-19 Vaccines; Humans; Purpura, Thrombocytopenic, Idiopathic; Purpura, Thrombotic Thrombocytopenic; SARS-CoV-2; Thrombosis; Vaccination
PubMed: 35259252
DOI: 10.1182/blood.2022015545 -
BMJ Case Reports Jan 2021
Topics: Female; Humans; Middle Aged; Pigmentation Disorders; Pruritus; Purpura
PubMed: 33462064
DOI: 10.1136/bcr-2020-240052 -
Hematology. American Society of... Dec 2017Thrombotic microangiopathies (TMAs) are a diverse group of disorders that are characterized by common clinical and laboratory features. The most commonly thought-of TMA... (Review)
Review
Thrombotic microangiopathies (TMAs) are a diverse group of disorders that are characterized by common clinical and laboratory features. The most commonly thought-of TMA is thrombotic thrombocytopenic purpura (TTP). Because of the marked improvement in patient mortality associated with the use of therapeutic plasma exchange (TPE) in TTP, this therapy has been applied to all of the TMAs. The issue, however, is that the pathophysiology varies and in many instances may represent a disorder of the endothelium and not the blood; in some cases, the pathophysiology is unknown. The use of TPE is further obscured by a lack of strong supporting literature on its use, with most consisting of case series and case reports; controlled or randomized controlled trials are lacking. Evidence supporting the use of TPE in the treatment of TMAs (other than TTP and TMA-complement mediated) is lacking, and therefore its role is uncertain. With the greater availability of genetic testing for mutations involving complement regulatory genes and complement pathway components, there seems to be a percentage of TMA cases, other than TMA-complement mediated, in which complement pathway mutations are involved in some patients. The ability of TPE to remove abnormal complement pathway components and replace them with normal components may support its use in some patients with TMAs other than TTP and TMA-complement mediated.
Topics: Complement Activation; Complement System Proteins; Humans; Mutation; Plasma Exchange; Purpura, Thrombotic Thrombocytopenic; Randomized Controlled Trials as Topic; Signal Transduction
PubMed: 29222314
DOI: 10.1182/asheducation-2017.1.632 -
Cleveland Clinic Journal of Medicine Jan 2024
Topics: Humans; Endocarditis, Bacterial; Endocarditis; Purpura
PubMed: 38167396
DOI: 10.3949/ccjm.91a.23041 -
Blood Advances May 2023Immune thrombotic thrombocytopenic purpura (iTTP) is an acquired, fatal microangiopathy if untreated. Randomized controlled trials (RCTs) demonstrated faster time to... (Meta-Analysis)
Meta-Analysis
Immune thrombotic thrombocytopenic purpura (iTTP) is an acquired, fatal microangiopathy if untreated. Randomized controlled trials (RCTs) demonstrated faster time to response with addition of caplacizumab to standard of care (SOC). However, concerns about RCT selection bias and the high cost of caplacizumab warrant examination of all evidence, including real-world observational studies. In this systematic review and meta-analysis, we searched for comparative studies evaluating SOC with or without caplacizumab for the treatment of iTTP. We assessed risk of bias using the Cochrane risk-of-bias-2 tool (RCTs) and the Newcastle-Ottawa Scale (observational studies). The primary efficacy and safety outcomes were all-cause mortality and treatment-emergent bleeding, respectively. Secondary outcomes included exacerbation and relapse, refractory iTTP, and time to response. We included 2 high-quality RCTs and 3 observational studies at high risk of bias comprising 632 total participants. Compared with SOC, caplacizumab was associated with a nonsignificant reduction in the relative risk [RR] of death in RCTs (RR, 0.21; 95% confidence interval [CI], 0.05-1.74) and observational studies (RR, 0.62; 95% CI, 0.07-4.41). Compared with SOC, caplacizumab was associated with an increased bleeding risk in RCTs (RR, 1.37; 95% CI, 1.06-1.77). In observational studies, bleeding risk was not significantly increased (RR, 7.10; 95% CI, 0.90-56.14). Addition of caplacizumab was associated with a significant reduction in refractory iTTP and exacerbation risks and shortened response time but increased relapse risk. Frontline addition of caplacizumab does not significantly reduce all-cause mortality compared with SOC alone, although it reduces refractory disease risk, shortens time to response, and improves exacerbation rates at the expense of increased relapse and bleeding risk.
Topics: Humans; Purpura, Thrombotic Thrombocytopenic; Standard of Care; Neoplasm Recurrence, Local; Hemorrhage; Purpura, Thrombocytopenic, Idiopathic
PubMed: 36053773
DOI: 10.1182/bloodadvances.2022008443 -
Disease-a-month : DM Oct 2014
Review
Topics: Antibodies, Monoclonal, Murine-Derived; Antineoplastic Agents; Disease Management; Humans; Immunosuppression Therapy; Immunotherapy; Plasma Exchange; Prognosis; Purpura, Thrombotic Thrombocytopenic; Risk Factors; United States
PubMed: 25278278
DOI: 10.1016/j.disamonth.2014.08.005 -
Clinics in Dermatology 2021Skin is one of target organs affected by the novel coronavirus SARS-CoV-2, and in response to the current COVID-19 pandemic, a fast body of literature has emerged on... (Review)
Review
Skin is one of target organs affected by the novel coronavirus SARS-CoV-2, and in response to the current COVID-19 pandemic, a fast body of literature has emerged on related cutaneous manifestations. Current perspective is that the skin is not only a bystander of the general cytokines storm with thrombophilic multiorgan injury, but it is directly affected by the epithelial tropism of the virus, as confirmed by the detection of SARS-CoV-2 in endothelial cells and epithelial cells of epidermis and eccrine glands. In contrast with the abundance of epidemiologic and clinical reports, histopathologic characterization of skin manifestations is limited. Without an adequate clinicopathologic correlation, nosology of clinically similar conditions is confusing, and effective association with COVID-19 remains presumptive. Several patients with different types of skin lesions, including the most specific acral chilblains-like lesions, showed negative results at SARS-CoV-2 nasopharyngeal and serologic sampling. The aim of this review is to provide an overview of what has currently been reported worldwide, with a particular emphasis on microscopic patterns of the skin manifestations in patients exposed to or affected by COVID-19. Substantial breakthroughs may occur in the near future from more skin biopsies, improvement of immunohistochemistry studies, RNA detection of SARS-CoV-2 strain by real-time polymerase chain reaction-based assay, and electron microscopic studies.
Topics: COVID-19; Chilblains; Erythema Multiforme; Exanthema; Humans; Necrosis; Purpura; SARS-CoV-2; Skin; Skin Diseases; Systemic Inflammatory Response Syndrome; Urticaria
PubMed: 33972045
DOI: 10.1016/j.clindermatol.2020.12.004 -
American Journal of Hematology Feb 2021Immune thrombocytopenia (ITP) is now well-known to reduce patients' health-related quality of life. However, data describing which signs and symptoms patients and...
Immune thrombocytopenia (ITP) is now well-known to reduce patients' health-related quality of life. However, data describing which signs and symptoms patients and physicians perceive as having the greatest impact are limited, as is understanding the full effects of ITP treatments. I-WISh (ITP World Impact Survey) was an exploratory, cross-sectional survey designed to establish the multifaceted impact of ITP, and its treatments, on patients' lives. It focused on perceptions of 1507 patients and 472 physicians from 13 countries regarding diagnostic pathway, frequency and severity of signs and symptoms, and treatment use. Twenty-two percent of patients experienced delayed diagnosis (caused by several factors), 73% of whom felt anxious as a result. Patients rated fatigue among the most frequent, severe symptom associated with ITP at diagnosis (58% most frequent; 73% most severe), although physicians assigned it lower priority (30%). Fatigue was one of the few symptoms persisting at survey completion (50% and 65%, respectively) and was the top symptom patients wanted resolved (46%). Participating physicians were experienced at treating ITP, thereby recognizing the need to limit corticosteroid use to newly-diagnosed or first-relapse patients and espoused increased use of thrombopoietin receptor agonists and anti-CD20 after relapse in patients with persistent/chronic disease. Patient and physicians were largely aligned on diagnosis, symptoms, and treatment use. I-WISh demonstrated that patients and physicians largely align on overall ITP symptom burden, with certain differences, for example, fatigue. Understanding the emotional and clinical toll of ITP on the patient will facilitate shared decision-management, setting and establishment of treatment goals and disease stage-appropriate treatment selection.
Topics: Adult; Aged; Chronic Disease; Cross-Sectional Studies; Female; Humans; Male; Middle Aged; Purpura, Thrombocytopenic, Idiopathic; Quality of Life; Surveys and Questionnaires
PubMed: 33170956
DOI: 10.1002/ajh.26045 -
Blood Aug 2019Insights into immune-mediated thrombotic thrombocytopenic purpura (iTTP) pathophysiology have led to novel targeted therapies. Immunomodulatory strategies target... (Review)
Review
Insights into immune-mediated thrombotic thrombocytopenic purpura (iTTP) pathophysiology have led to novel targeted therapies. Immunomodulatory strategies target anti-ADAMTS13 antibodies: rituximab is effective in inducing responses in refractory/relapsed TTP and increasing relapse-free survival; caplacizumab targets the von Willebrand factor-platelet interaction to hasten platelet count recovery and reduce mortality and TTP-related ischemic events. Bortezomib and recombinant ADAMTS13 are under investigation. This review examines how targeted therapies are disrupting current treatment paradigms to improve outcomes of iTTP.
Topics: Biomarkers; Clinical Decision-Making; Disease Management; Drug Interactions; Humans; Molecular Targeted Therapy; Prognosis; Purpura, Thrombotic Thrombocytopenic; Risk Assessment; Treatment Outcome
PubMed: 31217190
DOI: 10.1182/blood.2019000954