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La Revue Du Praticien Jan 2023PURPURA FULMINANS IN ADULT PATIENTS. Purpura fulminans is a rare life-threatening infectious disease characterized by the association of a sudden and extensive purpuric...
PURPURA FULMINANS IN ADULT PATIENTS. Purpura fulminans is a rare life-threatening infectious disease characterized by the association of a sudden and extensive purpuric rash together with an acute circulatory failure. PF commonly affects young patients with no previous comorbidities. Neisseria meningitidis and Streptococcus pneumoniae are the leading causative bacteria. Diagnosing purpura fulminans before the apparition of the purpuric rash is challenging since prodromal symptoms are nonspecific and consistent with a "flu-like" syndrome. The clinical presentation of patients with purpura fulminans differs from that of patients with bacterial meningitis since most of the patients with purpura fulminans have no neurological impairment. Microbiological diagnosis relies on blood cultures and skin biopsy of purpuric lesions. The indication for lumbar puncture must be evaluated on a case-by-case basis because patients usually have no neurological signs but severe coagulation disorders. Treatment is no different from that of any other septic shock: antibiotic therapy with a third-generation cephalosporin as soon as the diagnosis is suspected and treatment of associated organ failures. Despite these pathogens being highly susceptible to broadly available antibiotics, the prognosis of PF is dismal with a mortality rate of 40% in the intensive care unit and a significant risk of distant sequelae in surviving patients.
Topics: Humans; Adult; Purpura Fulminans; Skin; Neisseria meningitidis; Anti-Bacterial Agents; Exanthema
PubMed: 36820463
DOI: No ID Found -
Blood Reviews Sep 2021Immune thrombocytopenia (ITP) is a heterogeneous disease with an unpredictable course. Chronicity can develop in up to two-thirds of adults and 20-25% of children,... (Review)
Review
Immune thrombocytopenia (ITP) is a heterogeneous disease with an unpredictable course. Chronicity can develop in up to two-thirds of adults and 20-25% of children, representing a significant burden on patients' quality of life. Despite acceptable responses to treatment, precise etiology and pathophysiology phenomena driving evolution to chronicity remain undefined. We analyzed reported risk factors for chronic ITP and associated them with proposed underlying mechanisms in its pathogenesis, including bone marrow (BM) microenvironment disturbances, clinical features, and immunological markers. Their understanding has diagnostic implications, such as screening for the presence of specific antibodies or BM examination employing molecular tools, which could help predict prognosis and recognize main pathogenic pathways in each patient. Identifying these underlying mechanisms could guide the use of personalized therapies such as all-trans retinoic acid, mTor inhibitors, FcRn inhibitors, oseltamivir, and others. Further research should lead to tailored treatments and chronic course prevention, improving patients' quality of life.
Topics: Animals; Bone Marrow; Chronic Disease; Disease Management; Humans; Precision Medicine; Purpura, Thrombocytopenic, Idiopathic; Risk Factors
PubMed: 33771403
DOI: 10.1016/j.blre.2021.100827 -
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 -
Thrombosis Research Dec 2020Platelets play such an important role in the process of thrombosis that patients with thrombocytopenia generally have an increased risk of bleeding. However, abnormal... (Review)
Review
Platelets play such an important role in the process of thrombosis that patients with thrombocytopenia generally have an increased risk of bleeding. However, abnormal thrombotic events can sometimes occur in patients with thrombocytopenia, which is unusual and inexplicable. The treatments for thrombocytopenia and thromboembolism are usually contradictory. This review introduces the mechanisms of thromboembolism in patients with different types of thrombocytopenia and outlines treatment recommendations for the prevention and treatment of thrombosis. According to the cause of thrombocytopenia, this article addresses four etiologies, including inherited thrombocytopenia (Myh9-related disease, ANKRD26-associated thrombocytopenia, Glanzmann thrombasthenia, Bernard-Soulier syndrome), thrombotic microangiopathy (thrombotic thrombocytopenic purpura, atypical hemolytic uremic syndrome, hemolytic uremic syndrome, Hemolysis Elevated Liver enzymes and Low Platelets syndrome, disseminated intravascular coagulation), autoimmune-related thrombocytopenia (immune thrombocytopenic purpura, antiphospholipid syndrome, systemic lupus erythematosus), and acquired thrombocytopenia (Infection-induced thrombocytopenia and drug-induced thrombocytopenia, heparin-induced thrombocytopenia). We hope to provide more evidence for clinical applications and future research.
Topics: Disseminated Intravascular Coagulation; Humans; Purpura, Thrombocytopenic, Idiopathic; Purpura, Thrombotic Thrombocytopenic; Thromboembolism; Thrombotic Microangiopathies
PubMed: 33091704
DOI: 10.1016/j.thromres.2020.10.005 -
Chest May 2022Hematologic conditions (malignant or benign) may progress to acute critical illness requiring prompt recognition and intensive management. This review outlines... (Review)
Review
Hematologic conditions (malignant or benign) may progress to acute critical illness requiring prompt recognition and intensive management. This review outlines diagnostic considerations and approaches to management for intensivists of common benign hematologic emergencies, including the following: thrombotic thrombocytopenic purpura, atypical hemolytic uremic syndrome, disseminated intravascular coagulopathy, catastrophic antiphospholipid antibody syndrome, hemophagocytic lymphohistiocytosis, acute chest syndrome associated with sickle cell disease, and hyperhemolysis syndrome.
Topics: Adult; Critical Illness; Diagnosis, Differential; Emergencies; Hematology; Humans; Purpura, Thrombotic Thrombocytopenic
PubMed: 35007552
DOI: 10.1016/j.chest.2021.12.650 -
British Journal of Haematology Dec 2023Immune thrombotic thrombocytopenic purpura (iTTP) is an ultra-rare disease that seldom occurs in the elderly. Few reports have studied the clinical course of iTTP in...
Immune thrombotic thrombocytopenic purpura (iTTP) is an ultra-rare disease that seldom occurs in the elderly. Few reports have studied the clinical course of iTTP in older patients. In this study, we have analysed the clinical characteristics at presentation and response to therapy in a series of 44 patients with iTTP ≥60 years at diagnosis from the Spanish TTP Registry and compared them with 209 patients with <60 years at diagnosis from the same Registry. Similar symptoms and laboratory results were described in both groups, except for a higher incidence of renal dysfunction among older patients (23% vs. 43.1%; p = 0.008). Front-line treatment in patients ≥60 years was like that administered in younger patients. Also, no evidence of a difference in clinical response and overall survival was seen in both groups. Of note, 14 and 25 patients ≥60 years received treatment with caplacizumab and rituximab, respectively, showing a favourable safety and efficacy profile, like that observed in patients <60 years.
Topics: Humans; Aged; Purpura, Thrombotic Thrombocytopenic; Purpura, Thrombocytopenic, Idiopathic; Rituximab; Thrombosis; Plasma Exchange; Registries; ADAMTS13 Protein
PubMed: 37723363
DOI: 10.1111/bjh.19109 -
Hamostaseologie Aug 2021Immune thrombocytopenia (ITP) is an autoimmune disease that is characterized by a significant reduction in the number of circulating platelets and frequently associated... (Review)
Review
Immune thrombocytopenia (ITP) is an autoimmune disease that is characterized by a significant reduction in the number of circulating platelets and frequently associated with bleeding. Although the pathogenesis of ITP is still not completely elucidated, it is largely recognized that the low platelet count observed in ITP patients is due to multiple alterations of the immune system leading to increased platelet destruction as well as impaired thrombopoiesis. The clinical manifestations and patients' response to different treatments are very heterogeneous suggesting that ITP is a group of disorders sharing common characteristics, namely, loss of immune tolerance toward platelet (and megakaryocyte) antigens and dysfunctional primary hemostasis. Management of ITP is challenging and requires intensive communication between patients and caregivers. The decision to initiate treatment should be based on the platelet count level, age of the patient, bleeding manifestation, and other factors that influence the bleeding risk in individual patients. In this review, we present recent data on the mechanisms that lead to platelet destruction in ITP with a particular focus on current findings concerning alterations of thrombopoiesis. In addition, we give an insight into the efficacy and safety of current therapies and management of ITP bleeding emergencies.
Topics: Blood Platelets; Humans; Megakaryocytes; Platelet Count; Purpura, Thrombocytopenic, Idiopathic; Thrombopoiesis
PubMed: 33348391
DOI: 10.1055/a-1311-8264 -
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 -
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