RESUMEN
INTRODUCTION: Lung transplantation (LT) is the standard of care for patients with advanced lung diseases, including lymphangioleiomyomatosis (LAM). LAM accounts for only 1% of all LTs performed in the international registry. As a result, the global experience, including the use of mechanistic target of rapamycin (mTOR) inhibitors before and after LT in LAM, is still limited. METHODS: We conducted a retrospective review of all LAM patients who underwent LT at our centre between 2003 and 2016. Pre- and post-transplant data were assessed. RESULTS: Eleven women with LAM underwent LT, representing 3.3% of all procedures. Ten (91%) patients underwent double-LT. The mean age at diagnosis was 39 ± 6 years and the mean FEV1 before LT was 28 ± 14%. Only one patient underwent pleurodesis for recurrent pneumothorax. Pulmonary hypertension was confirmed in 3 (27%) patients. Four (36%) patients received sirolimus preoperatively; three of them received it until the day of LT, and there was no occurrence of bronchial anastomotic dehiscence after the procedure. Four patients (36%) received mTOR inhibitors post-transplant. The median follow-up from LT was 44 months. There were 3 deaths (27%) during the study and survival probabilities at 1, 3, and 5 years after LT were, 90, 90, and 77%, respectively. CONCLUSIONS: This data reinforces the role of LT for LAM patients with end-stage disease. The use of sirolimus seems to be safe before LT and the occurrence of complications after LT, including those LAM-related, should be continuously monitored.
Asunto(s)
Neoplasias Pulmonares/cirugía , Trasplante de Pulmón , Linfangioleiomiomatosis/cirugía , Adulto , Brasil , Everolimus/uso terapéutico , Femenino , Humanos , Inmunosupresores/uso terapéutico , Trasplante de Pulmón/efectos adversos , Persona de Mediana Edad , Estudios Retrospectivos , Sirolimus/uso terapéutico , Tasa de Supervivencia , Serina-Treonina Quinasas TOR/antagonistas & inhibidores , Centros de Atención Terciaria , Resultado del Tratamiento , Prueba de PasoRESUMEN
BACKGROUND: Lymphangioleiomyomatosis (LAM), a rare cystic disease characterized by proliferation of smooth muscle cells in the lung interstitium, almost exclusively affects females in their reproductive years. Lung transplantation has been established as effective therapy for end-stage pulmonary LAM. METHODS: This retrospective study includes lung transplantation patients with LAM at a single institution between 1989 and 2009. RESULTS: During the study period we performed 300 lung transplantations, and in 10 cases the recipients had LAM. All patients were females with a mean age of 43.8 years. The mean time from the diagnosis to lung transplantation was 5 years. Seven patients had experienced previous pneumothoraces, five of whom were treated with pleurodesis. In all patients we performed a single-lung transplantation (left-sided = 9 and right-sided = 1). In three cases, the pleurodesis was on the same side as the transplantation, with great intraoperative bleeding in one subject (left pleurectomy). There was one early death due to infective endocarditis at posttransplant day 19. The median length of mechanical ventilation was 13 hours, while the mean hospital stay was 16.75 days. There was no case of chylothorax. Late complications included one case of native lung pneumothorax, one diaphragmatic hernia, one posttransplant lymphoproliferative disease, one respiratory sepsis, and one mycobacterial infection. The 1- and 3-year survival rates were 90% and 80%, respectively. CONCLUSION: Lung transplantation is a feasible therapeutic option for patients with LAM, despite previous ipsilateral pleurodesis. The left-sided predilection for our procedures may have been responsible for the absence of chylothorax in this series.
Asunto(s)
Trasplante de Pulmón , Linfangioleiomiomatosis/cirugía , Adulto , Brasil , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios RetrospectivosAsunto(s)
Enfermedades Bronquiales/cirugía , Trasplante de Pulmón/efectos adversos , Stents , Adulto , Enfermedades Bronquiales/etiología , Constricción Patológica/etiología , Constricción Patológica/cirugía , Resultado Fatal , Femenino , Humanos , Neoplasias Pulmonares/cirugía , Linfangioleiomiomatosis/cirugía , SiliconasAsunto(s)
Adulto , Femenino , Humanos , Enfermedades Bronquiales/cirugía , Trasplante de Pulmón/efectos adversos , Stents , Enfermedades Bronquiales/etiología , Constricción Patológica/etiología , Constricción Patológica/cirugía , Resultado Fatal , Neoplasias Pulmonares/cirugía , Linfangioleiomiomatosis/cirugía , SiliconasAsunto(s)
Neoplasias Pulmonares , Linfangioleiomiomatosis , Adulto , Biopsia , Diagnóstico Diferencial , Femenino , Humanos , Pulmón/patología , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/diagnóstico por imagen , Neoplasias Pulmonares/tratamiento farmacológico , Neoplasias Pulmonares/patología , Neoplasias Pulmonares/cirugía , Linfangioleiomiomatosis/diagnóstico , Linfangioleiomiomatosis/diagnóstico por imagen , Linfangioleiomiomatosis/tratamiento farmacológico , Linfangioleiomiomatosis/patología , Linfangioleiomiomatosis/cirugía , Ovariectomía , Progesterona/uso terapéutico , Radiografía Torácica , Tomografía Computarizada por Rayos XAsunto(s)
Humanos , Femenino , Adulto , Neoplasias Pulmonares , Linfangioleiomiomatosis , Biopsia , Diagnóstico Diferencial , Linfangioleiomiomatosis/diagnóstico , Linfangioleiomiomatosis/tratamiento farmacológico , Linfangioleiomiomatosis/patología , Linfangioleiomiomatosis , Linfangioleiomiomatosis/cirugía , Ovariectomía , Progesterona/uso terapéutico , Pulmón/patología , Radiografía Torácica , Tomografía Computarizada por Rayos XRESUMEN
Se presenta el caso de una paciente de 36 años, que ingresa al hospital cursando un embarazo fisiológico de 32 semanas, por cuadro hemoptisis masivas (> 700 ml/24 horas) asociadas a disnea, con leve inestabilidad hemodinámica. La fibrobroncoscopía mostró sangrando proveniente del segmento posterior del lóbulo superior derecho, sin daño endoluminal. La radiografía de tórax, tomografía y angiografía bronquial fueron normales. La paciente persistió con sangramiento como fue confirmado en una segunda broncospía por lo que optó por la resolución quirúrgica efectuándose una lobectomía superior derecha. El análisis histopatológico de la pieza concluyó el diagnóstico de Linfagioleiomiomatosis (LAM) y el estudio para receptores de estrógenos fue positivo en el tejido analizado. La LAM es una entidad rara de origen desconocido que afecta sólo a mujeres, especialmente en edad fértil, caracterizada por proliferación del músculo lisi a nivel peribronquial, perivascular y perilifática que conduce a la formación de lesiones quísticas, neumotórax recurrente, hemoptisis y quilotórax. La historia natural de la LAM es hacia la progresiva obstrucción al flujo aéreo, que conduce la falla respiratoria progresiva en un plazo impredecible. Las series más recientes comunican una sobrevida a 8-10 años de 79 por ciento. La hipótesis de la dependencia hormonal y efectividad de terapias tales como ooforectomía y progesterona no han sido demostradas en forma sólida, sin embargo, en la actualidad son las alternativas disponibles previo a la decisión de transplante pulmonar