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European Journal of Haematology Dec 2022In late February 2021, a prothrombotic syndrome was encountered for the first time in some of the recipients of ChAdOx1 CoV-19 vaccine (AstraZeneca, University of... (Review)
Review
In late February 2021, a prothrombotic syndrome was encountered for the first time in some of the recipients of ChAdOx1 CoV-19 vaccine (AstraZeneca, University of Oxford, and Serum Institute of India). Since the hallmark of this syndrome is the development of thrombocytopenia and/or thrombosis between 4 and 42 days after receiving a COVID-19 vaccine, it was named vaccine-induced immune thrombotic thrombocytopenia (VITT). Other names include "vaccine-induced prothrombotic immune thrombocytopenia" and "thrombosis with thrombocytopenia syndrome" by the Centers for Disease Control and the Food and Drug Administration (FDA). VITT appears similar to heparin-induced thrombocytopenia in that "platelet activating" autoantibodies are produced in both these conditions due to prior exposure of COVID-19 vaccine and heparin respectively, in turn causing thrombotic complications and consumptive thrombocytopenia. In this article, recent advances in the understanding of pathobiology, clinical features, investigative work-up, and management of VITT are reviewed.
Topics: Humans; COVID-19; COVID-19 Vaccines; Purpura, Thrombocytopenic, Idiopathic; Thrombocytopenia; Thrombosis; Vaccines
PubMed: 36030503
DOI: 10.1111/ejh.13855 -
Blood Dec 2022
Topics: Humans; Qi; Purpura, Thrombocytopenic, Idiopathic; Decitabine; Myeloid-Derived Suppressor Cells; Thrombocytopenia
PubMed: 36580341
DOI: 10.1182/blood.2022018373 -
Hamostaseologie Feb 2024
Topics: Humans; Purpura, Thrombocytopenic, Idiopathic; Thrombocytopenia; Thrombosis
PubMed: 38417798
DOI: 10.1055/s-0044-1782593 -
Tidsskrift For Den Norske Laegeforening... May 2024
Topics: Humans; Purpura; Golf
PubMed: 38738575
DOI: 10.4045/tidsskr.24.0262 -
Ugeskrift For Laeger May 2020In this review, we discuss pigmented purpuric dermatoses (PPD), which are a group of benign, chronic diseases characterised by purpuric eruption. PPD comprise mb.... (Review)
Review
In this review, we discuss pigmented purpuric dermatoses (PPD), which are a group of benign, chronic diseases characterised by purpuric eruption. PPD comprise mb. Schamberg, mb. Majocchi, Gougerot-Blum, lichen aureus, and Doucas and Kapetanakis eczematoid purpura. PPD can be seen in both genders and may affect all age groups. Purpura is often localised to the lower extremities, and it may be asymptomatic or pruritic. PPD is usually diagnosed upon recognition of classical clinical features, but the diagnosis can also be confirmed by a skin biopsy.
Topics: Female; Humans; Keratosis; Male; Pigmentation Disorders; Pruritus; Purpura; Skin
PubMed: 32515323
DOI: No ID Found -
International Journal of Hematology Mar 2023Immune thrombocytopenia (ITP), thrombotic thrombocytopenic purpura (TTP), and vaccine-induced immune thrombotic thrombocytopenia (VITT) all have "thrombocytopenia" in... (Review)
Review
Immune thrombocytopenia (ITP), thrombotic thrombocytopenic purpura (TTP), and vaccine-induced immune thrombotic thrombocytopenia (VITT) all have "thrombocytopenia" in their name, and all but congenital TTP are caused by immune mechanisms, but these conditions are quite different in their clinical features and pathophysiology. This review series covers recent progress in pathophysiology and treatment of these diseases, as well as a recent epoch-making clinical trial of induced pluripotent stem cells (iPSC)-derived platelets for patients with thrombocytopenia.
Topics: Humans; Purpura, Thrombotic Thrombocytopenic; Purpura, Thrombocytopenic, Idiopathic; Blood Platelets
PubMed: 36656456
DOI: 10.1007/s12185-023-03542-w -
Blood Sep 2021
Topics: Humans; Immunoglobulins, Intravenous; Platelet Factor 4; Purpura, Thrombocytopenic, Idiopathic
PubMed: 34529019
DOI: 10.1182/blood.2021012819 -
Clinical Medicine (London, England) May 2023Acute thrombosis and thrombocytopenia pose challenges to the clinician. Thrombocytopenia is naturally viewed as a risk factor for bleeding, and an association with acute... (Review)
Review
Acute thrombosis and thrombocytopenia pose challenges to the clinician. Thrombocytopenia is naturally viewed as a risk factor for bleeding, and an association with acute thrombosis appears paradoxical. It presents typically as a medical emergency and requires treatment to be started before having confirmatory results. This review supports the attending clinician to recognise and manage conditions that are part of the thrombotic thrombocytopenic syndrome through four illustrative clinical cases. Common themes linking the underlying pathology and treatment are explored to highlight the continued relevance of this rare, but often devastating, presentation.
Topics: Humans; Purpura, Thrombotic Thrombocytopenic; Thrombosis; Syndrome; Risk Factors
PubMed: 37236794
DOI: 10.7861/clinmed2023-0076 -
Cold Spring Harbor Molecular Case... Feb 2022Ethylmalonic encephalopathy (MIM #602473) is a rare autosomal recessive metabolic condition caused by biallelic variants in (MIM #608451), characterized by global...
Ethylmalonic encephalopathy (MIM #602473) is a rare autosomal recessive metabolic condition caused by biallelic variants in (MIM #608451), characterized by global developmental delay, infantile hypotonia, seizures, and microvascular damage. The microvascular changes result in a pattern of relapsing spontaneous diffuse petechiae and purpura, positional acrocyanosis, and pedal edema, hemorrhagic suffusions of mucous membranes, and chronic diarrhea. Here, we describe an instructive case in which ethylmalonic encephalopathy masqueraded as meningococcal septicemia and shock. Ultrarapid whole-genome testing (time to result 60 h) and prompt biochemical analysis facilitated accurate diagnosis and counseling with rapid implementation of precision treatment for the metabolic crisis related to this condition. This case provides a timely reminder to consider rare genetic diagnoses when atypical features of more common conditions are present, with an early referral to ensure prompt biochemical and genomic diagnosis.
Topics: Brain Diseases, Metabolic, Inborn; Humans; Mitochondrial Proteins; Nucleocytoplasmic Transport Proteins; Purpura; Sepsis
PubMed: 35165146
DOI: 10.1101/mcs.a006193 -
International Journal of Molecular... Sep 2022Coronavirus disease 2019 (COVID-19) can lead to clinically significant multisystem disorders that also affect the kidney. According to recent data, renal injury in the... (Review)
Review
Coronavirus disease 2019 (COVID-19) can lead to clinically significant multisystem disorders that also affect the kidney. According to recent data, renal injury in the form of thrombotic microangiopathy (TMA) in native kidneys ranks third in frequency. Our review of global literature revealed 46 cases of TMA in association with COVID-19. Among identified cases, 18 patients presented as thrombotic thrombocytopenic purpura (TTP) and 28 cases presented as atypical hemolytic uremic syndrome (aHUS). Altogether, seven patients with aHUS had previously proven pathogenic or likely pathogenic genetic complement abnormalities. TMA occurred at the time of viremia or even after viral clearance. Infection with COVID-19 resulted in almost no or only mild respiratory symptoms in the majority of patients, while digestive symptoms occurred in almost one-third of patients. Regarding the clinical presentation of COVID-19-associated TMA, the cases showed no major deviations from the known presentation. Patients with TTP were treated with plasma exchange (88.9%) or fresh frozen plasma (11.1%), corticosteroids (88.9%), rituximab (38.9%), and caplacizumab (11.1%). Furthermore, 53.6% of patients with aHUS underwent plasma exchange with or without steroid as initial therapy, and 57.1% of patients received a C5 complement inhibitor. Mortality in the studied cohort was 16.7% for patients with TTP and 10.7% for patients with aHUS. The exact role of COVID-19 in the setting of COVID-19-associated TMA remains unclear. COVID-19 likely represents a second hit of aHUS or TTP that manifests in genetically predisposed individuals. Early identification of the TMA subtype and appropriate prompt and specific treatment could lead to good outcomes comparable to survival and recovery statistics for TMA of all causes.
Topics: Atypical Hemolytic Uremic Syndrome; COVID-19; Complement Inactivating Agents; Humans; Purpura, Thrombotic Thrombocytopenic; Rituximab; Steroids; Thrombotic Microangiopathies
PubMed: 36232608
DOI: 10.3390/ijms231911307