National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Plummer Vinson syndrome


Other Names:
Kelly's syndrome; Paterson-Kelly syndrome; Paterson-Brown-Kelly syndrome; Kelly's syndrome; Paterson-Kelly syndrome; Paterson-Brown-Kelly syndrome; Dysphagia sideropenica; Paterson’s syndrome; Kelly-Paterson syndrome; Plummer-Vinson syndrome; Sideropenic dysphagia See More
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The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.
orphanet

Orpha Number: 54028

Definition
Plummer-Vinson or Paterson-Kelly syndrome presents as a classical triad of dysphagia, iron-deficiency anemia and esophageal webs.

Epidemiology
Exact data about the epidemiology of the syndrome are not available; the syndrome is extremely rare.

Clinical description
Most of the patients are white middle-aged women, in the fourth to seventh decade of life, but the syndrome has also been described in children and adolescents. The dysphagia is usually painless and intermittent or progressive over years, limited to solids and sometimes associated with weight loss. Symptoms resulting from anemia (weakness, pallor, fatigue, tachycardia) may dominate the clinical picture. Additional features are glossitis, angular cheilitis and koilonychia. Enlargement of the spleen and thyroid may also be observed. One of the most important clinical aspects of Plummer-Vinson syndrome is the association with upper alimentary tract cancers.

Etiology
The etiopathogenesis of Plummer-Vinson syndrome is unknown. The most important possible etiological factor is iron deficiency. Other possible factors include malnutrition, genetic predisposition or autoimmune processes.

Management and treatment
Plummer-Vinson syndrome can be treated effectively with iron supplementation and mechanical dilation. In case of significant obstruction of the esophageal lumen by esophageal web and persistent dysphagia despite iron supplementation, rupture and dilation of the web are necessary. Since Plummer-Vinson syndrome is associated with an increased risk of squamous cell carcinoma of the pharynx and the esophagus, the patients should be followed closely.

Visit the Orphanet disease page for more resources.
Last updated: 9/1/2006

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms Other Names
Learn More:
HPO ID
100% of people have these symptoms
Decreased serum ferritin
Low ferritin level
Reduced serum ferritin
[ more ] 		0012343
Dysphagia
Poor swallowing
Swallowing difficulties
Swallowing difficulty
[ more ] 		0002015
Esophageal web 0100594
Hypochromic microcytic anemia 0004840
Iron deficiency anemia 0001891
80%-99% of people have these symptoms
Easy fatigability 0003388
Glossitis
Inflammation of the tongue
Smooth swollen tongue
[ more ] 		0000206
Pallor 0000980
5%-29% of people have these symptoms
Abdominal pain
Pain in stomach
Stomach pain
[ more ] 		0002027
Cheilitis
Inflammation of the lips
0100825
Concave nail
Spoon-shaped nails
0001598
Geophagia 0025062
Intra-oral hyperpigmentation 0010284
Narrow mouth
Small mouth
0000160
Poor appetite
Decreased appetite
0004396
Tongue atrophy
Wasting of the tongue
0012473
Showing of 16 |
Last updated: 7/1/2020

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Plummer Vinson syndrome. Click on the link to view a sample search on this topic.

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