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.
Subject(s)
Humans , Adult , Female , Anemia, Iron-Deficiency , Esophageal Spasm, Diffuse , Plummer-Vinson Syndrome/diagnosis , Deglutition DisordersABSTRACT
A síndrome de Plummer-Vinson é caracterizada por disfagia cervical, deficiência de ferro e presença de membrana esofágica. Neste estudo, relatam-se dois casos dessa síndrome em irmãos adolescentes. Eles não obtiveram aumento dos níveis hematimétricos após reposição com ferro oral, o que, associado à eletroforese de hemoglobinas, sustentou o diagnóstico de talassemia concomitante. Devido ao quadro dos filhos, os pais foram também submetidos à eletroforese de hemoglobinas cujo diagnóstico do pai foi talassemia alfa/beta menor e da mãe, talassemia alfa menor. Os irmãos tiveram disfagia refratária e necessitaram de dilatação endoscópica. Ambos necessitaram de terapia com ferro venoso com melhora dos níveis hematimétricos.
Plummer-Vinson syndrome is characterized by cervical dysphagia, iron deficiency and the presence of esophageal membranes. We report two cases of this syndrome present in adolescent brothers with associated thalassemia. After oral iron therapy, their hematimetric levels showed no increase, which associated with the results of hemoglobin electrophoresis, sustained the diagnosis of thalassemia. Due to the condition of the children, the parents were submitted to hemoglobin electrophoresis examinations; the father was diagnosed as having minor alpha/beta thalassemia and the mother as minor beta thalassemia. Both patients suffered from refractory dysphagia and required endoscopic dilatation. They both underwent venous iron therapy, which improved the hematimetric levels.