Asunto(s)
Dermatitis Exfoliativa , Dermatomiositis , Micosis Fungoide , Miositis , Neoplasias Cutáneas , Humanos , Dermatitis Exfoliativa/diagnóstico , Dermatitis Exfoliativa/etiología , Dermatomiositis/complicaciones , Dermatomiositis/diagnóstico , Micosis Fungoide/complicaciones , Micosis Fungoide/diagnósticoAsunto(s)
Resistencia a Antineoplásicos/genética , Eliminación de Gen , Imidazoles/efectos adversos , Sarcoma de Células de Langerhans/tratamiento farmacológico , Mutación , Oximas/efectos adversos , Proteínas Proto-Oncogénicas B-raf/antagonistas & inhibidores , Proteínas Proto-Oncogénicas B-raf/genética , Proteína Activadora de GTPasa p120/genética , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Azetidinas/administración & dosificación , Humanos , Sarcoma de Células de Langerhans/patología , MAP Quinasa Quinasa 1/antagonistas & inhibidores , Masculino , Persona de Mediana Edad , Recurrencia Local de Neoplasia/inducido químicamente , Recurrencia Local de Neoplasia/tratamiento farmacológico , Recurrencia Local de Neoplasia/patología , Piperidinas/administración & dosificación , Pronóstico , Inhibidores de Proteínas Quinasas/efectos adversos , Vemurafenib/administración & dosificaciónRESUMEN
Lung transplantation (LTx) is the last-resort treatment for end-stage respiratory insufficiency, whatever its origin, and represents a steadily expanding field of endeavor. Major developments have been impelled over the years by painstaking efforts at LTx centers to improve donor and recipient selection, and multifaceted attempts have been made to meet the challenges raised by surgical management, perioperative care, and long-term medical complications. The number of procedures has increased, leading to improved post-LTx prognosis. One consequence of these multiple developments has been a pruning away of contraindications over time, which has, in some ways, complicated the patient selection process. With these considerations in mind, the Francophone Pulmonology Society (Société de Pneumology de Langue Française [SPLF]) has set up a task force to produce up-to-date working guidelines designed to assist pulmonologists in managing end-stage respiratory insufficiency, determining which patients may be eligible for LTx, and appropriately timing LTx-center referral. The task force has examined the most recent literature and evaluated the risk factors that continue to limit patient survival after LTx. Ideally, the objectives of LTx are to prolong life while improving quality of life. The guidelines developed by the task force apply to a limited resource and are consistent with the ethical principles described below.
Asunto(s)
Trasplante de Pulmón , Insuficiencia Respiratoria , Humanos , Calidad de Vida , Trasplante de Pulmón/métodos , Francia/epidemiología , Contraindicaciones , Insuficiencia Respiratoria/etiologíaAsunto(s)
Antineoplásicos/uso terapéutico , Resistencia a Antineoplásicos/efectos de los fármacos , Imidazoles/uso terapéutico , Sarcoma de Células de Langerhans/tratamiento farmacológico , Oximas/uso terapéutico , Terapia Recuperativa/tendencias , Antineoplásicos/farmacología , Humanos , Imidazoles/farmacología , Sarcoma de Células de Langerhans/diagnóstico por imagen , Sarcoma de Células de Langerhans/genética , Masculino , Persona de Mediana Edad , Oximas/farmacología , Proteínas Proto-Oncogénicas B-raf/genética , Resultado del TratamientoAsunto(s)
Anticuerpos Monoclonales de Origen Murino/uso terapéutico , Bronquiolitis Obliterante/tratamiento farmacológico , Trasplante de Células Madre Hematopoyéticas , Factores Inmunológicos/uso terapéutico , Adolescente , Adulto , Bronquiolitis Obliterante/diagnóstico , Bronquiolitis Obliterante/cirugía , Broncodilatadores/uso terapéutico , Resultado Fatal , Femenino , Enfermedad Injerto contra Huésped/diagnóstico , Enfermedad Injerto contra Huésped/tratamiento farmacológico , Humanos , Inmunosupresores , Macrólidos/uso terapéutico , Masculino , Ácido Micofenólico/análogos & derivados , Ácido Micofenólico/uso terapéutico , Prednisona/uso terapéutico , Rituximab , Tacrolimus/uso terapéutico , Acondicionamiento Pretrasplante , Resultado del TratamientoRESUMEN
Langerhans cell histiocytosis (LCH) is a rare disease characterized by the infiltration of one or more organs by Langerhans cell-like dendritic cells, most often organized in granulomas. The disease has been initially described in children. The clinical picture of LCH is highly variable. Bone, skin, pituitary gland, lung, central nervous system, lymphoid organs are the main organs involved whereas liver and intestinal tract localizations are less frequently encountered. LCH course ranges from a fulminant multisystem disease to spontaneous resolution. Several randomized controlled trials have enable pediatricians to refine the management of children with LCH. Adult LCH has some specific features and poses distinct therapeutic challenges, knowing that data on these patients are limited. Herein, we will provide an overview of current knowledge regarding adult LCH and its management. We will also discuss recent advances in the understanding of the disease, (i.e. the role of BRAF oncogene) that opens the way toward targeted therapies.
Asunto(s)
Histiocitosis de Células de Langerhans , Adulto , Edad de Inicio , Diagnóstico Diferencial , Histiocitosis de Células de Langerhans/diagnóstico , Histiocitosis de Células de Langerhans/epidemiología , Histiocitosis de Células de Langerhans/genética , Histiocitosis de Células de Langerhans/terapia , Humanos , Neoplasias/epidemiología , Neoplasias/etiologíaRESUMEN
Bronchiolitis obliterans syndrome (BOS) after allogeneic hematopoietic SCT (HSCT) is recognized as a new-onset obstructive lung defect (OLD) in pulmonary function testing and is related to pulmonary chronic GVHD. Little is known about the different phenotypes of patients with BOS and their outcomes. We reviewed the data of all allogeneic HSCT recipients referred to our pulmonary department for a non-infectious bronchial disease between 1999 and 2010. We identified 103 patients (BOS (n=77), asthma (n=11) and chronic bronchitis (n=15)). In patients with BOS, we identified two functional phenotypes: a typical OLD, that is, forced expiratory volume in 1 s (FEV1)/forced vital capacity (FVC) ratio <0.7 (n=53), and an atypical OLD with a concomitant decrease in the FEV1 <80% and FVC <80% predicted with a normal total lung capacity (n=24). The typical OLD was characterized by more severe FEV1 and fewer centrilobular nodules on the computed tomography scan. The FEV1 was not significantly affected during the follow-up, regardless of the phenotype. In addition to acute and extensive chronic GVHD, only the occurrence of BOS soon after transplantation and the intentional treatment of BOS with steroids were associated with a poor survival. The determination of patient subgroups should be explored to improve the management of this condition.