Acute respiratory failure in adult patients with acute myeloid leukemia.

Introduction: Patients with acute myeloid leukemia (AML) are at high risk of developing life-threatening complications. It is estimated that a quarter of adult patients diagnosed with AML will require admission to the intensive care unit (ICU) at least once during their disease. Acute respiratory failure (ARF) is the main reason for ICU admission and is associated with high mortality rates, depending on the etiology of ARF.

Pulmonary Langerhans cell histiocytosis - an update on pathogenesis and treatment.

Purpose Of Review: Pulmonary Langerhans cell histiocytosis (PLCH) is a rare diffuse cystic lung disease that affects young to middle-aged smoking adults of both genders. The identification of molecular alterations in the canonical mitogen-activated protein kinase (MAPK) signalling pathway in most specific lesions has demonstrated the clonal/neoplastic nature of PLCH. We will summarize the progress made in the understanding of the pathogenesis of adult PLCH, and briefly highlight the recent findings useful for the management of the patients.

[Respiratory manifestations of Ehlers-Danlos syndromes].

Ehlers-Danlos syndromes (EDS) represent a heterogeneous group of heritable connective tissue disorders characterized by the clinical "triad" consisting in joint hypermobility, skin hyperextensibility and tissue fragility. Respiratory manifestations associated with EDS are frequent and variable. They vary mainly according to the type of EDS.

Long-term outcomes of adult pulmonary Langerhans cell histiocytosis: a prospective cohort.

Background: The long-term outcomes of adult pulmonary Langerhans cell histiocytosis (PLCH), particularly survival, are largely unknown. Two earlier retrospective studies reported a high rate of mortality, which contrasts with our clinical experience.

Methods: To address this issue, all patients with newly diagnosed PLCH referred to the French national reference centre for histiocytoses between 2004 and 2018 were eligible for inclusion.