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Blood Jan 2023
Topics: Humans; Purpura, Thrombotic Thrombocytopenic; Plasma Exchange; Recurrence; Chronic Disease
PubMed: 36656610
DOI: 10.1182/blood.2022019297 -
International Journal of Molecular... Feb 2024Pigmented purpuric dermatoses (PPD) encompass a group of chronic skin conditions characterized by the presence of petechiae, purpura, and pigmentation changes. While... (Review)
Review
Pigmented purpuric dermatoses (PPD) encompass a group of chronic skin conditions characterized by the presence of petechiae, purpura, and pigmentation changes. While generally benign, these dermatoses can be persistent and aesthetically bothersome. Key clinical features include red to brownish patches with a distinctive "cayenne pepper" appearance, predominantly localized on the lower extremities, particularly the shins. Subtypes include Schamberg disease, Majocchi's disease, Gougerot-Blum disease, Ducas and Kapetanakis pigmented purpura, and lichen aureus. Diagnosis relies primarily on clinical evaluation of skin lesions, with biopsy as a confirmatory tool. Although the exact cause of PPD remains unclear, capillary fragility and red blood cell extravasation are implicated. Treatment strategies for PPD aim to alleviate symptoms, considering the generally benign and chronic nature of the condition. As there is no standardized treatment, various methods with varying efficacy are employed. After searching SCOPUS and PubMed databases, we assessed 42 original articles to present current knowledge regarding therapy of PPD. This review will compare treatment approaches specifically in Schamberg disease and other manifestations of pigmented purpuric dermatoses.
Topics: Humans; Pigmentation Disorders; Skin Diseases; Purpura; Eczema; Vascular Diseases
PubMed: 38473891
DOI: 10.3390/ijms25052644 -
Emergencias : Revista de La Sociedad... Feb 2023Acquired or immune thrombotic thrombocytopenic purpura (TTP) are thrombotic microangiopathies associated with high mortality if treatment is not started early. Onset is...
Immune thrombotic thrombocytopenic purpura: clinical suspicion and basic management in emergency departments - an expert review and consensus statement from the Spanish societies of hematology and hemotherapy (SEHH) and emergency medicine (SEMES).
Acquired or immune thrombotic thrombocytopenic purpura (TTP) are thrombotic microangiopathies associated with high mortality if treatment is not started early. Onset is usually sudden, meaning that the condition is often diagnosed in hospital emergency departments, where TTP must be suspected as early as possible. These guidelines were drafted by specialists in emergency medicine and hematology to cover the diagnosis, referral, and treatment of patients suspected of immune-mediated TTP who require emergency care. Immune TTP should be suspected whenever a patient presents with hemolytic microangiopathy and has a negative Coombs test, and thrombocytopenia, possibly in conjunction with fever and neurologic and cardiac alterations. If one of the existing diagnostic algorithms indicates there is a high probability that the patient has immune TTP, plasma exchange therapy should be started along with immunosuppressants. Treatment with caplacizumab should also be considered. The patient should be referred immediately to the hematology department within the same hospital or a referral hospital.
Topics: Humans; Emergency Medicine; Emergency Service, Hospital; Hematology; Plasma Exchange; Purpura, Thrombotic Thrombocytopenic
PubMed: 36756916
DOI: No ID Found -
Journal of Thrombosis and Haemostasis :... Sep 2022The diagnosis of immune thrombocytopenia (ITP) is one of exclusion. Although guidelines recommend against routine bone marrow examination (BME) at time of ITP diagnosis,...
BACKGROUND
The diagnosis of immune thrombocytopenia (ITP) is one of exclusion. Although guidelines recommend against routine bone marrow examination (BME) at time of ITP diagnosis, the role of BME in relapsed/refractory ITP is unclear.
OBJECTIVES
To examine the frequency and predictors of BME in relapsed/refractory ITP.
PATIENTS/METHODS
This multicenter retrospective cohort study included adults with ITP who received second-line therapy in Alberta, Canada from 2012 to 2019. We calculated the frequency of BME and rate of abnormal marrow findings. Logistic regression was performed to assess predictors of BME and predictors of bone marrow pathology.
RESULTS
Of 324 patients with presumed ITP, 181 (56%) underwent BME. We observed a marked decline in the rates of BME among patients >60 years over the past decade, but not in patients younger than age 60 years. On multivariable logistic regression, older age (adjusted OR [aOR] 1.03, p = .0001), anemia (aOR 2.5, p = .01), splenomegaly (aOR 3.2, p = .01), splenectomy (aOR 2.4, p = .02), and lack of splenectomy response (aOR 3.4, p = .04) were significant predictors of BME. Abnormal marrow findings were found in eight (2% of overall cohort; 4% of BME): four myelodysplastic syndrome, one aplastic anemia, one chronic lymphocytic leukemia, one metastatic cancer, and one megaloblastic anemia. Seven (88%) underwent BME for bicytopenias/pancytopenias. Macrocytosis (aOR 9.6, p = .03) and rural residence (aOR 6.7, p = .02) were independent predictors of abnormal bone marrow findings.
CONCLUSIONS
Although routine BME is frequently performed in relapsed/refractory ITP, abnormal findings are rare. Future prospective studies are needed to help identify a subgroup of relapsed/refractory ITP who may benefit from BME.
Topics: Adult; Anemia, Aplastic; Bone Marrow Examination; Humans; Middle Aged; Purpura, Thrombocytopenic, Idiopathic; Retrospective Studies; Thrombocytopenia
PubMed: 35751575
DOI: 10.1111/jth.15802 -
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 -
Medicina Clinica Jan 2023
Topics: Humans; Purpura, Thrombocytopenic, Idiopathic; COVID-19 Vaccines; COVID-19; Platelet Count; Vaccination
PubMed: 36030095
DOI: 10.1016/j.medcli.2022.06.023 -
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 -
Acta Medica Portuguesa Feb 2021COVID-19 is an infectious disease caused by the new coronavirus, SARS-CoV-2, that has spread rapidly throughout the world. This has resulted in an urgent need to obtain... (Review)
Review
INTRODUCTION
COVID-19 is an infectious disease caused by the new coronavirus, SARS-CoV-2, that has spread rapidly throughout the world. This has resulted in an urgent need to obtain information regarding its pathogenesis, diagnosis and clinical manifestations. More specifically, skin manifestations, seldom reported initially, have been increasingly described.
MATERIAL AND METHODS
We performed a literature search in the PubMed database, regarding cutaneous manifestations associated with COVID-19. This article describes the clinical and histological findings of the main skin lesions observed in the context of SARS-CoV-2 infection.
DISCUSSION
Cutaneous manifestations associated with COVID-19 have been described in multiple retrospective and prospective studies, case series and case reports. The reported incidence reached 20.4%. Although there was substantial heterogeneity in terms of clinical patterns, the main ones include: erythematous/maculopapular, urticarial, papulovesicular, and purpuric/petechial eruptions, chilblain-like lesions and livedoid/acro-ischemic lesions. In the vast majority, the underlying pathophysiologic mechanisms are not fully understood, although histopathological findings and biomolecular studies can add relevant data.
CONCLUSION
The recognition of cutaneous manifestations associated with COVID-19 is of utmost importance. They may help establishing an early diagnosis, namely in oligosymptomatic patients or when confirmatory tests are impossible to perform. Moreover, chilblain-like lesions and acro-ischemia, also seem to play an important role in terms of prognosis.
Topics: COVID-19; Erythema; Humans; Incidence; Ischemia; Nicolau Syndrome; Prospective Studies; Purpura; Retrospective Studies; SARS-CoV-2; Skin Diseases, Viral; Toes; Urticaria
PubMed: 33315008
DOI: 10.20344/amp.14574 -
Blood Jun 2023Antibody binding to a plasma metalloprotease, a disintegrin and metalloproteinase with thrombospondin type 1 repeats 13 (ADAMTS13), is necessary for the development of...
Antibody binding to a plasma metalloprotease, a disintegrin and metalloproteinase with thrombospondin type 1 repeats 13 (ADAMTS13), is necessary for the development of immune thrombotic thrombocytopenic purpura (iTTP). Inhibition of ADAMTS13-mediated von Willebrand factor (VWF) cleavage by such antibodies clearly plays a role in the pathophysiology of the disease, although the mechanisms by which they inhibit ADAMTS13 enzymatic function are not fully understood. At least some immunoglobulin G-type antibodies appear to affect the conformational accessibility of ADAMTS13 domains involved in both substrate recognition and inhibitory antibody binding. We used single-chain fragments of the variable region previously identified via phage display from patients with iTTP to explore the mechanisms of action of inhibitory human monoclonal antibodies. Using recombinant full-length ADAMTS13, truncated ADAMTS13 variants, and native ADAMTS13 in normal human plasma, we found that, regardless of the conditions tested, all 3 inhibitory monoclonal antibodies tested affected enzyme turnover rate much more than substrate recognition of VWF. Hydrogen-to-deuterium exchange plus mass spectrometry experiments with each of these inhibitory antibodies demonstrated that residues in the active site of the catalytic domain of ADAMTS13 are differentially exposed to solvent in the presence and absence of monoclonal antibody binding. These results support the hypothesis that inhibition of ADAMTS13 in iTTP may not necessarily occur because the antibodies directly prevent VWF binding, but instead because of allosteric effects that impair VWF cleavage, likely by affecting the conformation of the catalytic center in the protease domain of ADAMTS13. Our findings provide novel insight into the mechanism of autoantibody-mediated inhibition of ADAMTS13 and pathogenesis of iTTP.
Topics: Humans; Purpura, Thrombotic Thrombocytopenic; Antibodies, Monoclonal; von Willebrand Factor; ADAM Proteins; Thrombosis; Purpura, Thrombocytopenic, Idiopathic; ADAMTS13 Protein; Autoantibodies
PubMed: 37023370
DOI: 10.1182/blood.2022019252 -
Contrast Media & Molecular Imaging 2022The purpose of this study was to investigate the absolute value of peripheral blood lymphocytes in patients with primary immune thrombocytopenia and the diagnostic...
The purpose of this study was to investigate the absolute value of peripheral blood lymphocytes in patients with primary immune thrombocytopenia and the diagnostic effect on patients with primary immune thrombocytopenia. From January 2020 to June 2021, 76 patients with primary immune thrombocytopenia and 80 healthy check-ups admitted to our hospital were selected as study subjects and divided into a control group (80 patients, healthy check-ups) and an observation group (76 patients, primary immune thrombocytopenia), according to the health status of the organism. Early morning fasting venous blood was collected from both groups, and the absolute value of peripheral blood lymphocytes was measured and compared using a fully automated hematology analyzer to investigate the diagnostic value of absolute peripheral blood lymphocytes in primary immune thrombocytopenia. The CD3+, CD3+CD4+, CD4+/CD8+, and CD16+CD56+ assay values in the observation group were lower than those in the control group, and the CD3+CD8+, CD19+, and ALC assay values were higher than those in the control group ( < 0.05). The CD3+CD8+ detection values of newly diagnosed patients were similar to those of relapsed refractory patients ( > 0.05); CD3+, CD3+CD4+, CD4+/CD8+, and CD16+CD56+ detection values of newly diagnosed patients were lower than those of relapsed refractory patients, and CD19+ and ALC detection values were higher than those of relapsed refractory patients; CD3+, CD3+CD4+, CD4+, CD4+/CD8+, and CD16+CD56+ detection values of mild patients were lower than those of relapsed refractory patients; CD3+, CD3+CD4+, CD4+/CD8+, and CD16+CD56+ detection values were higher in mild patients than in severe patients, and CD3+CD8+, CD19+, and ALC detection values were lower than in severe patients ( < 0.05). The absolute lymphocyte values were of high diagnostic value in primary immune thrombocytopenia, with a sensitivity and specificity of 93.42% and 90.00%. The application of absolute peripheral blood lymphocyte value in the clinical diagnosis of primary immune thrombocytopenia can achieve a better detection and diagnosis effect, which has a positive impact on the early diagnosis rate and can help patients to obtain more timely, effective and targeted treatment, and is worthy of promotion.
Topics: Humans; Lymphocytes; Purpura, Thrombocytopenic, Idiopathic
PubMed: 36072617
DOI: 10.1155/2022/9833941