ABSTRACT
Resumen El síndrome de Plummer-Vinson (Paterson-Brown-Kelly) es una entidad rara, caracterizada por disfagia, formación de membranas esofágicas y anemia por deficiencia de hierro. Presentamos el caso de una mujer de 46 años con antecedentes clínicos de anemia ferropénica de larga evolución, que posteriormente presentó disfagia y odinofagia. Se encontró una membrana subcricoidea que fue rota exitosamente con el endoscopio. Los médicos de primer contacto deben estar familiarizados de los síntomas del síndrome de Plummer- Vinson, y tenerlos en cuenta a la hora de abordar un paciente con la tríada clásica. Como el síndrome es una condición pre- cancerosa con alto potencial maligno, el diagnóstico precoz y tratamiento oportuno es de suma importancia.
Abstract Plummer-Vinson syndrome (Paterson-Brown-Kelly) is a rare entity, characterized by dysphagia, esophageal web formation, and iron deficiency anemia. We present the case of a 46-year-old woman with a clinical history of iron deficiency anemia who subsequently presents dysphagia and odynophagia. A subcricoid web that was successfully broken with the endoscope was found. First contact doctors should be familiar with the symptoms of Plummer-Vinson syndrome, and take them into account, when addressing a patient with the classic triad. Since the syndrome is a precancerous condition with high malignant potential, early diagnosis and treatment is of utmost importance for better prognosis.
ABSTRACT
Presentamos a una mujer de 39 años que acude a nuestro hospital por disnea de esfuerzo, adinamia y cansancio progresivo desde hace 8 meses. Antecedente quirúrgico de miomectomia por menometrorragias frecuentes. Niega ser vegetariana. Régimen catamenial: 3-5 días cada mes. Hace 2 meses se añade ôardorõ en lengua al comer. Nota uñas quebradizas. Toleraba alimentos blandos. Al examen físico había moderada palidez; las uñas eran muy delgadas, frágiles y algo cóncavas. En la boca se encontró estomatitis angular, lengua depapilada y glositis. El diagnóstico clínico era síndrome anémico y disfagia. Exámenes auxiliares: Hb: 7.0g/dL; VCM: 57.42fL; HCM: 15.82pg; leucocitos: 4,980; reticulocitos: 2.18%, índice reticulocitario: 0.1%, hierro sérico: 21ug/dl, transferrina (TIBC): 286, saturación de transferrina: 7%, ferritina sérica: 27ng/ml. La lámina periférica demostró anisocitosis, hipocromía y microcitosis. Thevenon en heces negativo. Ecografía abdominal: mioma uterino. La radiografía esofágica con bario demostró una imagen lineal por defecto de relleno de 2 mm a altura entre vértebras C4 y C5 en vista anteroposterior y lateral; se extendía a cara anterior y reducía la luz esofágica en 60%. Durante la endoscopía, evidenciamos una membrana fibrosa estenosante en la región cricofaríngea. Multiples dilataciones fueron realizadas progresivamente mediante una guía con bujías dilatadoras Savary-Gilliard hasta 14 mm, mejorando la disfagia. Recibió terapia transfusional y hierro parenteral. Salió de alta con sulfato ferroso y ácido fólico. El síndrome de Plummer-Vinson, Paterson-Brown-Kelly o disfagia sideropénica es definido por disfagia, anemia ferropénica y membrana esofágica alta. El síndrome es descrito como muy raro.
A 39-year-old woman was admitted to our hospital with an eight-month history of dyspnea on exertion, weakness and increasing fatigue. She reported repeated episodes of menome trorrhsgis snd underwent a myomectomy. She is not a vegetarian. Her menstrual bleeding: 3-5 days per month. Two months ago, she complained of burning sensation of the tonge upon swallowing food and noted brittle nails. She tolerated soft foods. On physical examination, she was pale; her nails were very thin, fragile and somewhat concave. Her oral examination showed angular stomatitis, depapillated togue and glossitis. The clinical diagnosis was anemia and dysphagia. Laboratory tests were: Hb: 7.0g/dL, MCV: 57.42fL, MCH: 15.82 pg; leukocytes:4,980; reticulocytes:2.18%, reticulocyte index:0.1%, serum iron:21ug/dl, total iron binding capacity (TIBC):286, transferrin saturation: 7% and serum ferritin: 27ng/ml. The peripheral blood smear showed anisocytosis and hypochromic microcytic cells. Thevenon test was negative. Abdominal ultrasound: uterine myoma. A barium swallow X-ray showed a 2-mm linear filling defect between the 4th and 5th cervical vertebrae in the anteroposterior and lateral view; it protruded from the anterior wall and reduced esophageal lumen by 60%. In the endoscopy, we found a fibrous web in the cricopharyngeal area. Serial dilatations were performed over a guidewire using Savary-Gilliard dilators with diameter up to 14 mm, improving dysphagia. She was treated with transfusional therapy and parenteral iron. She was discharged with ferrous sulfate and folic acid. The Plummer-Vinson syndrome, Paterson-Brown-Kelly or sideropenic dysphagia is characterized by dysphagia, iron-deficiency anemia and upper esophaegal web. The syndrome is described as very rare.