Plummer–Vinson syndrome

Plummer–Vinson syndrome

The triad of iron deficiency anemia, dysphagia, and a cervical esophageal web is known as Plummer-Vinson syndrome or Paterson-Brown-Kelly syndrome. Patients with Plummer-Vinson syndrome are typically white women in their fourth to seventh decade of life, but it has also been described in children and adolescents. While in the past Plummer-Vinson syndrome was thought to be common in Caucasians in northern countries, it is now extremely rare, possibly due to a fall in the prevalence of iron deficiency anemia in these countries over time. Recognition of Plummer-Vinson syndrome is important because it is a risk factor for developing esophageal or pharyngeal squamous cell carcinoma.

Other findings that may be present in patients with Plummer-Vinson syndrome include glossitis, angular cheilitis, koilonychia, splenomegaly, an enlarged thyroid, and symptoms of iron deficiency anemia (eg, fatigue and weakness). It is diagnosed based on the finding of iron deficiency anemia on laboratory testing and by demonstration of an esophageal web on barium esophagram, videofluoroscopy, or upper endoscopy.

In patients with Plummer-Vinson syndrome, iron repletion may lead to rapid resolution of the dysphagia before hematologic abnormalities are corrected. However, in patients with significant obstruction of the esophageal lumen, esophageal dilation may also be required. Annual surveillance upper endoscopy for esophageal squamous cell carcinoma has also been suggested by some experts, although this has not been shown to improve patient outcomes.

 


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