ABSTRACT
Pulmonary Langerhans'-cell histiocytosis belongs to a spectrum of diseases characterized by monoclonal proliferation and infiltration of organs by Langerhans' cells. It is an uncommon interstitial lung disease of unknown etiology occurring almost exclusively in cigarette smokers. It s course in adults is variable and unpredictable, ranging from benign self-limiting types with spontaneous regression to slowly progressive malignant disease that leads to respiratory failure and death. We report one patient diagnoses of pulmonary Langerhans' cell histiocytosis who experimented an objective radiographic improvement and disappearance of symptoms after smoking cessation.
Subject(s)
Histiocytosis, Langerhans-Cell/diagnosis , Adult , Humans , Lung/diagnostic imaging , Lung/pathology , Male , Radiography , Remission, Spontaneous , Smoking CessationABSTRACT
La histiocitosis pulmonar de células de Langerhans forma parte de un espectro de enfermedades caracterizadas por la proliferación monoclonal y la infiltración de distintos órganos por células de Langerhans. Es una enfermedad pulmonar intersticial de etiología desconocida que ocurre casi exclusivamente en pacientes fumadores. El curso de esta enfermedad en adultos es impredecible, oscilando desde formas benignas autolimitadas, hasta formas malignas con evolución progresiva hacia el fallo respiratorio y la muerte. Presentamos el caso de un paciente diagnosticado de histiocitosis pulmonar de células de Langerhans que presentó una mejoría clínica y radiográfica tras abandonar el hábito tabáquico
Pulmonary Langerhans´- cell histiocytosis belongs to a spectrum of diseases characterized by monoclonal proliferation and infiltration of organs by Langerhans´cells. It is an uncommon interstitial lung disease of unknown etiology occurring almost exclusively in cigarette smokers. It´s course in adults is variable and unpredictable, ranging from benign self-limiting types with spontaneous regression to slowly progressive malignant disease that leads to respiratory failure and death. We report one patient diagnoses of pulmonary Langerhans´cell histiocytosis who experimented an objetive radiographic improvement and disappearance of symptoms after smoking cessation
Subject(s)
Male , Adult , Humans , Histiocytosis, Langerhans-Cell/pathology , Histiocytosis, Langerhans-Cell/etiology , Tobacco Use Disorder/complications , Tomography, X-Ray ComputedABSTRACT
Whipple's disease is a rare systemic infectious disease caused by the bacterium Tropheryma whippelii. Early diagnosis is essential. Whipple's disease is potentially fatal but responds dramatically to antibiotic treatment. The diagnosis is confirmed by means of polymerase chain reaction (PCR) technology. This analysis may be useful for monitoring the efficacy of therapy. The recommended treatment al present is administration of cotrimoxazole twice daily for one year. When CNS involvement occurs, it is recommended initial treatment with daily parenteral administration of streptomycin 1 g and 1.2 million units of benzyl penicillin (Penicillin G) over a period of 14 days.
Subject(s)
Whipple Disease/diagnosis , Aged , Anemia/etiology , Arthritis, Infectious/diagnosis , Arthritis, Infectious/etiology , Biopsy , DNA, Bacterial/isolation & purification , Diarrhea/etiology , Duodenal Diseases/etiology , Duodenal Diseases/microbiology , Duodenal Diseases/pathology , Female , Folic Acid/therapeutic use , Gastrointestinal Hemorrhage/etiology , Gram-Positive Bacteria/genetics , Gram-Positive Bacteria/isolation & purification , Heart Failure/etiology , Heart Valve Diseases/etiology , Heart Valve Diseases/surgery , Humans , Laparotomy , Male , Penicillin G/therapeutic use , Polymerase Chain Reaction , Streptomycin/therapeutic use , Trimethoprim, Sulfamethoxazole Drug Combination/therapeutic use , Weight Loss , Whipple Disease/drug therapyABSTRACT
La enfermedad de Whipple, también conocida como lipodistrofia intestinal, es un proceso infeccioso, multisistémico y poco frecuente, causado por la bacteria Tropheryma whippelii. El diagnóstico precoz es fundamental porque esta enfermedad es potencialmente letal, pero responde espectacularmente al tratamiento antibiótico. El diagnóstico se confirma con la reacción en cadena de la polimerasa (PCR) que también es útil para monitorizar la respuesta al tratamiento. Actualmente el tratamiento recomendado consiste en la administración de cotrimoxazol oral dos veces al día durante un año. Cuando hay afectación del SNC se aconseja iniciar el tratamiento con la administración intramuscular de 1 gramo de estreptomicina asociado a 1,2 MU de bencil penicilina (penicilina G) de forma diaria durante 14 días
Whipples disease is a rare systemic infectious disease caused by the bacterium Tropheryma whippelii. Early diagnosis is essential. Whipples disease is potentially fatal but responds dramatically to antibiotic treatment. The diagnosis is confirmed by means of polymerase chain reaction (PCR) technology. This analysis may be useful for monitoring the efficacy of therapy. The recommended treatment al present is administration of cotrimoxazole twice daily for one year. When CNS involvement occurs, it is recommended initial treatment with daily parenteral administration of streptomycin 1 g and 1,2 million units of benzyl penicillin (Penicillin G) over a period of 14 days