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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 -
Harefuah Mar 2019Primary immune thrombocytopenic purpura (ITP) is an acquired immune-mediated disorder characterized by isolated thrombocytopenia (platelet count less than 100X109/L),... (Review)
Review
Primary immune thrombocytopenic purpura (ITP) is an acquired immune-mediated disorder characterized by isolated thrombocytopenia (platelet count less than 100X109/L), caused by IgG autoantibodies which bind to platelets and megakaryocyte, T cell-mediated platelet destruction and impaired megakaryocytic function. Symptoms can manifest as petechiae, purpura, mucosal bleeding and rarely fatal intracranial hemorrhage, as well as reduced quality of life. A wide range of bleeding manifestations exists and it is impossible to tell who will bleed, when and where. The goal of treatment is to prevent severe/life-threatening bleeding. Treatment modalities target various aspects of ITP pathophysiology such as the inhibition of autoantibody production (decreased autoimmune process), modulation of T cell activity (with prolongation of platelets survival), and stimulation of platelet production. The American Society of Hematology and the International Society of Thrombosis and Hemostasis published guidelines on the treatment of ITP patients, where first line treatment focuses on inhibition of autoantibody production and platelet degradation, second-line treatments include immunosuppressive drugs and splenectomy, and third-line treatments aim to stimulate platelet production by megakaryocytes. New available strategies might change the order of treatment lines. As in other situations, treatment should be tailored according to the patient's age, life style, comorbidities and compliance.
Topics: Adult; Blood Platelets; Humans; Megakaryocytes; Purpura, Thrombocytopenic, Idiopathic; Quality of Life; Thrombopoiesis
PubMed: 30916510
DOI: No ID Found -
The American Journal of Dermatopathology Feb 2015Rare clinical variants of bullous pemphigoid (BP) include vesicular BP, dyshidrosiform BP, pemphigoid nodularis, seborrheic BP, pemphigoid vegetans, localized BP,...
Rare clinical variants of bullous pemphigoid (BP) include vesicular BP, dyshidrosiform BP, pemphigoid nodularis, seborrheic BP, pemphigoid vegetans, localized BP, erythrodermic BP, and juvenile BP. To our knowledge, this is the first report of an unusual case of purpuric BP. We present a case of 85-year-old white man who presented with a 2-week history of blisters and pruritic urticarial lesions all over his body. The diagnosis of purpuric BP was made on the basis of history, clinical presentation, histopathology report, direct and indirect immunofluorescence studies the diagnosis of purpuric BP was made. The reason for the development of palmoplantar purpuric lesions concomitant to ordinary patches and plaques of BP is unknown.
Topics: Aged, 80 and over; Biopsy; Dapsone; Diagnosis, Differential; Drug Administration Schedule; Drug Therapy, Combination; Fluorescent Antibody Technique; Glucocorticoids; Humans; Male; Pemphigoid, Bullous; Predictive Value of Tests; Prednisolone; Purpura; Skin; Treatment Outcome
PubMed: 25607328
DOI: 10.1097/DAD.0000000000000057 -
JAMA Otolaryngology-- Head & Neck... Sep 2016Strangulation accounts for 10% of violent deaths in the United States and 15% to 20% of deaths associated with domestic violence. However, strangulation deaths represent... (Review)
Review
IMPORTANCE
Strangulation accounts for 10% of violent deaths in the United States and 15% to 20% of deaths associated with domestic violence. However, strangulation deaths represent only a small fraction of nonfatal strangulation assaults, which occur with daily frequency in medium to large US cities. Careful evaluation and documentation of strangulation injuries may identify life-threatening medical conditions, and may facilitate prosecution of strangulation crimes.
OBSERVATIONS
The most recent article on strangulation identified in the otolaryngology literature was published in 1989, leaving a generation of head and neck surgeons without current guidance regarding this injury. However, strangulation is a common form of intimate partner violence. Among the 300 cases of strangulation reviewed in San Diego in 1995, most survivors (94%) were women who were strangled by a male member of their own household. Many state laws require evidence of injury to prosecute felony strangulation, but as shown in the review of 300 cases, most survivors (97%) were strangled with the perpetrator's hands, leaving little to no sign of injury in most cases. Survivors may seek an otolaryngology consultation with complaints of hoarseness, sore throat, respiratory disturbance, or accidental injury to the neck. A thorough head and neck examination may reveal marks on the neck, facial petechiae, and neck swelling. Fiberoptic laryngoscopy is recommended to look for petechiae and swelling in the airway. Chest radiographs may demonstrate postobstructive edema, and computed tomography of the neck may demonstrate vascular injuries. The most sensitive test for subtle strangulation injuries is magnetic imaging of the neck. Careful examination and documentation can provide critical evidence for the prosecution of these crimes.
CONCLUSIONS AND RELEVANCE
Otolaryngologists should be knowledgeable of the subtle patterns of injury and potentially life-threatening complications of strangulation and should consider domestic violence in women presenting with throat complaints or bruises on the neck.
Topics: Asphyxia; Deglutition Disorders; Emergency Service, Hospital; Forensic Medicine; Hoarseness; Homicide; Humans; Hypoxia; Intimate Partner Violence; Laryngoscopy; Magnetic Resonance Imaging; Neck Injuries; Neck Pain; Pharyngitis; Physical Examination; Purpura; Survivors
PubMed: 27308820
DOI: 10.1001/jamaoto.2016.0293 -
Dermatologic Therapy Apr 2022
Topics: COVID-19; COVID-19 Vaccines; Humans; Purpura; Vaccination
PubMed: 35137500
DOI: 10.1111/dth.15360 -
Journal of Cosmetic Dermatology Jan 2023COVID-19 is a highly contagious respiratory tract infection caused by severe acute respiratory syndrome coronavirus 2. COVID-19 outbreak, which caused thousands of... (Review)
Review
BACKGROUND
COVID-19 is a highly contagious respiratory tract infection caused by severe acute respiratory syndrome coronavirus 2. COVID-19 outbreak, which caused thousands of deaths, has been declared a pandemic by the World Health Organization in March 2020.
AIM
Skin manifestations related to SARS-CoV-2 infection can be divided mainly into five groups: chilblainlike lesions (CBLLs), maculopapular eruptions, urticarial eruptions, vesicular eruptions, and livedo or necrosis. Other skin findings reported are erythema multiforme (EM)-like lesions and skin findings associated with multisystem inflammatory syndrome in children (MIS-C) and rarely with multisystem inflammatory syndrome in adults (MIS-A). Other manifestations such as pityriasis rosea or shingles are also reported.
METHODS
A total of 60 articles including reviews, studies and case reports were selected for the evaluation in this review.
RESULTS
The skin manifestations associated with COVID-19 infection are numerous and can vary widely. The major dermatological patterns of COVID-19 can be classified as inflammatory reactions (maculopapular/morbilliform, urticarial and vesicular rashes), or lesions of vascular origin (chilblain like rashes, petechiae/purpura, and livedo acemose-like pattern) CONCLUSION: We believe that the dermatologist could play an important role in the response to the SARS-CoV-2 pandemic through early recognition of skin lesions suggestive of COVID-19, particularly in paucisymptomatic infections where this recognition could direct toward an early diagnosis of infection that certainly leads to a better prognosis.
Topics: Adult; Child; Humans; COVID-19; SARS-CoV-2; Skin Diseases; Purpura
PubMed: 36342945
DOI: 10.1111/jocd.15477 -
Praxis Jul 2017
Topics: Aged; Diagnosis, Differential; Erythema; Female; Humans; Leg; Leg Dermatoses; Physical Exertion; Purpura; Vasculitis
PubMed: 28677489
DOI: 10.1024/1661-8157/a002724 -
JAMA Dermatology Aug 2021
Topics: Humans; Purpura
PubMed: 34190960
DOI: 10.1001/jamadermatol.2021.1792 -
International Journal of Dermatology Jun 2021
Topics: Erythema Infectiosum; Foot Dermatoses; Hand Dermatoses; Humans; Parvovirus B19, Human; Purpura; Skin Diseases
PubMed: 33565622
DOI: 10.1111/ijd.15456 -
Clinical and Experimental Dermatology Apr 2022Dermatologists should consider Achenbach syndrome in the differential diagnosis for patients with purpura on the fingers. The patient should be monitored following...
Dermatologists should consider Achenbach syndrome in the differential diagnosis for patients with purpura on the fingers. The patient should be monitored following appropriate examination and invasive tests, such as skin biopsy or angiography, should be avoided unless necessary.
Topics: Diagnosis, Differential; Fingers; Hematoma; Humans; Male; Purpura; Syndrome
PubMed: 34856004
DOI: 10.1111/ced.15042