La Revue de médecine interne
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[Atypical and/or systemic dermatologic disorders related to immune checkpoint inhibitors: A review].
Immunological checkpoint inhibitors are now part of the oncological therapeutic arsenal in many solid cancers and malignant blood diseases, at the cost of immuno-mediated toxicities, of which dermatological disorders are among the most frequent. The most common, following treatment with anti-PD1 or anti-CTLA4, are maculopapular erythema, pruritus, vitiligo, or lichenoid lesions, but other more atypical conditions may lead to the internist being called upon. Here, we present a case series of these less common dermatological manifestations including fasciitis, dermatomyositis, scleroderma, granulomatosis and immune-induced vasculitis. Some manifestations appear similar to the primary forms or seem to correspond to paraneoplastic syndromes, but some diagnostic and therapeutic particularities are specific to ICI toxicity that the internist must be aware of.
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Thrombotic Thrombocytopenic Purpura (TTP) is a rare disease characterized by a severe deficiency of ADAMTS13, the specific protease that cleaves von Willebrand factor. The congenital form of TTP (cTTP) results from pathogenic variants of the ADAMTS13 gene. cTTP has two peaks of incidence: one in childhood and the other in adulthood, mainly in an obstetric context. The treatment of cTTP relies on ADAMTS13 replacement therapy for prophylaxis or on-demand, depending on the evolving nature of the disease, along with the management of cardiovascular risk factors. ⋯ Since 2017, a recombinant human ADAMTS13 protein (rhADAMTS13) has been evaluated in cTTP in international clinical trials. The rhADAMTS13 protein, intravenous infusion used for prophylaxis or on-demand, has been granted early access or compassionate use in cTTP in France in 2024. The objective of this document is to establish academic recommendations for the use of rhADAMTS13 in cTTP.
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Delirium, also known as acute confusional state, is an acute brain disorder characterized by cognitive disturbances, particularly attentional deficits, potential behavioral changes, and altered vigilance, with a sudden onset and fluctuating course. It is a common condition among hospitalized older patients and has serious consequences for the patient, their family, and the healthcare system. ⋯ This narrative review aims to describe the symptoms that allow the diagnosis of delirium in older adults, the available diagnostic or screening tools, as well as the complex and bidirectional relationships between delirium and dementia. The management of delirium, including non-pharmacological measures, will be discussed, along with symptomatic pharmacological treatments, which should be reserved for severe cases despite their low level of evidence.