International protocol for the treatment of Plummer Vinson syndrome. What is Plummer's disease - the growth of nodules on the thyroid gland

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(D.R. Paterson, 1883-1939, English otorhinolaryngologist; A.V. Kelly, 1865-1941, Scottish otorhinolaryngologist)
see Plummer-Vinson syndrome.


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Plummer Vinson syndrome is defined by the classic triad of dysphagia, iron deficiency anemia and an esophageal problem. Although very rare, its diagnosis is important because it identifies a group of patients at increased risk for squamous cell carcinoma of the pharynx and esophagus.

  • Plummer-Vinson, P.V.S.;
  • Paterson-Kelly syndrome;
  • Paterson-Brown Kelly;
  • Sideropenic dysphagia.

Story

The most commonly used name is Plummer-Vinson syndrome. It is named after Henry Stanley Plummer (1874-1936) and Porter Paisley Vinson (1890-1959), who were physicians at the Mayo Clinic.

In 1912, Plummer reported a series of patients with perennial iron deficiency anemia, dysphagia, and upper esophageal spasm without anatomical stenosis, which was described as hysterical.

A synonym is Paterson-Kelly syndrome, named after Donald Ross Paterson (1863-1939) and Adam Brown-Kelly (1865-1941). British laryngologists who published their results independently in 1919.

They were the first to describe the characteristic clinical features of the syndrome. Paterson gave a full description, but without reference to anemia. He was also the first to note the association with postcricoid carcinoma. Brown-Kelly not only described the signs and symptoms of the condition, but also anemia.

Epidemiology

Accurate data on the prevalence of the syndrome are not available. In the first half of the 20th century, Plummer-Vinson syndrome seemed common among Caucasians in northern countries, especially among middle-aged women. Currently it is extremely rare.

Of 1000 patients who underwent cineradographic examination of the hypopharynx and cervical esophagus, webs were found in 5.5% of cases, but only six had dysphagia associated with Vinson syndrome.

The rapid decline in the prevalence of the syndrome correlates with improvements in nutrition and the disappearance of widespread iron deficiency in countries where it was previously described. In Africa, where iron deficiency and malnutrition are common, the syndrome is very rare.

Clinical description

The main clinical features of Plummer-Vinson syndrome are postcricoid dysphagia, upper esophageal networks, and iron deficiency anemia. Most patients are white, middle-aged women, in the fourth decade of life, but the syndrome also occurs in children and adolescents.

Dysphagia is usually painless, intermittent, progressive over years, limited to particulate matter, and sometimes associated with weight loss.

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Symptoms resulting from anemia, such as weakness, pallor, fatigue, and tachycardia, dominate the clinical picture. In addition, it is characterized by glossitis, angular cheilitis, koiloniches (spoon-shaped nails). Enlargement of the spleen and thyroid gland is also observed.

Plummer-Vinson syndrome is a risk factor for the development of squamous cell carcinoma of the upper gastrointestinal tract. It has been reported that 3 to 15 percent of patients with Plummer-Vinson syndrome, mostly women ages 15 to 50, develop cancer of the esophagus or pharynx.

A decreasing trend in the overall incidence of hypopharyngeal cancer in women has been demonstrated, likely due to a decrease in the prevalence of the syndrome.

Case reports

An analysis of case reports published in the literature over a 7-year period (1999-2005) showed that 25 of 28 adult patients with Plummer-Vinson syndrome were women (89%). The mean age at presentation was 47 years (range, 28–80 years).

All patients had iron deficiency anemia with a mean hemoglobin value of 8.2 g/dL. The most common reports were associated illness (n = 6), increased menstrual blood loss (n = 5), oral hernia, chronic gastrointestinal bleeding of unknown origin (n = 3).

Iron supplements were prescribed for all patients. The majority underwent esophageal dilatation (n = 20). Overall the prognosis was very good.


Patients remained free of dysphagia during the follow-up period. In two cases, squamous cell carcinoma was diagnosed; in one case, gastric cancer was detected by endoscopy one year after the diagnosis of Plummer-Vinson syndrome. This malignancy is extremely rare but has been described in two case reports in previous years.

Diagnostics

Diagnosis is based on evidence of iron deficiency anemia and one or more esophageal tissues in a patient with postcricoid dysphagia. Esophageal tissue can be detected using a barium x-ray, but videofluoroscopy is the best way to demonstrate it. Symptoms are detected by upper gastrointestinal endoscopy.

They appear smooth, thin, gray with an eccentric or central clearance. Meshes are usually found in the proximal esophagus. They can be missed and accidentally torn if the endoscope is not inserted under direct visualization.

Esophageal networks, which also occur in the absence of anemia and Plummer-Vinson syndrome, are characterized by one or more thin horizontal membranes consisting of squamous epithelium and submucosa. Usually protrude from the anterior wall, extending transversely. But not to the back wall, they rarely surround the lumen.

Laboratory tests

Laboratory studies usually reveal iron deficiency anemia with decreased values ​​of hemoglobin, hematocrit, serum iron, ferritin, and increased total iron binding capacity. No other laboratory abnormalities have been described.

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Differential diagnosis

Because dysphagia is the main clinical feature of Plummer-Vinson syndrome, the differential diagnosis includes all other causes of dysphagia, especially malignancy, benign strictures, or esophageal rings.

Other causes of dysphagia include diverticula, mobility disorders such as achalasia, spastic motility disorders, scleroderma, diabetes mellitus, neuromuscular, and skeletal muscle disorders.


Etiology and pathogenesis

The pathogenesis of Plummer-Vinson syndrome is unknown. The most important possible etiological factor is iron deficiency. This theory is based primarily on the fact that iron deficiency is part of the classic triad of Plummer-Vinson syndrome, dysphagia and esophageal tissue, and that dysphagia improves with iron supplementation.

Indeed, impaired esophageal motility is described in Plummer-Vinson syndrome and is compensated by iron supplementation. Iron deficiency has been shown to precede dysphagia. On the other hand, the digestive tract is susceptible to iron deficiency.

Rapidly loses iron-dependent enzymes due to high cellular turnover. It causes degeneration of the mucous membrane and the formation of tissue. However, large clinical series have shown that for many patients, iron deficiency is neither a necessary nor a sufficient cause of these formations.

Other etiological factors have been proposed, including malnutrition, genetic predisposition, and autoimmune processes. The latter is based on the association between Plummer-Vinson syndrome and certain autoimmune disorders. For example, celiac disease, thyroid disease, rheumatoid arthritis.

Treatment

The first step in treating Plummer-Vinson syndrome is to find out the cause of the iron deficiency. To rule out active hemorrhage, malignancy, or celiac disease.

Plummer-Vinson syndrome is easily and effectively treated with iron supplementation and mechanical dilatation. Iron alone can resolve dysphagia in many patients. However, if there is significant lumen or cavity obstruction of the esophagus and persistent dysphagia despite iron supplementation, tissue rupture and expansion should be performed.

After endoscopic placement of the guide wire, dilators up to 17 mm in diameter are used. Usually one dilatation is enough to relieve dysphagia, but sometimes multiple sessions are required. Successful balloon dilation has also been described.

Plummer-Vinson syndrome is a systemic disease. Its main manifestations are a decrease in hemoglobin associated with iron deficiency, atrophy of the mucous membrane of the digestive organs and impaired swallowing. In addition, patients also experience abnormalities in other organs and systems. Patients complain of poor condition of nails and skin, inflammation of the tongue, and blurred vision. This is a complex pathology that is difficult to treat. The disease occurs mainly in women aged 20 to 50 years.

Etiology

Medical research has not established the exact cause of Plummer-Vinson syndrome. It is assumed that this pathology occurs due to a deficiency of enzymes involved in iron metabolism. As a result, a deficiency of B9 (riboflavin and folic acid) is formed. And also the body begins to lack iron. A deficiency of this element leads to anemia, weakness of the muscles involved in the act of swallowing, and pathological changes in the epithelium of the esophagus and stomach.

Other reasons for the development of the disease may be poor nutrition, in which the diet does not contain enough foods with iron, as well as genetic predisposition.

Some medical scientists believe that this pathology is associated with autoimmune disorders. Plummer-Vinson syndrome often occurs in patients with rheumatoid arthritis, thyroiditis and gluten protein intolerance.

Symptoms

At the onset of the disease, patients complain of weakness, increased fatigue, and tiredness. They experience increased heart rate. These are the first signs of iron deficiency anemia. Next, systemic changes begin in many organs, which is expressed in the following symptoms:

  1. It becomes difficult for a person to swallow food, and pain occurs in the esophagus. At first, the patient cannot eat only rough food. But then difficulties arise even when swallowing soft foods and liquids.
  2. Due to mucosal atrophy, damage to the upper part of the esophagus occurs. Then gastritis and enteritis develop.
  3. The nails become thinner and take on a concave shape.
  4. The skin of the face becomes greasy, inflamed and covered with acne.
  5. Inflamed cracks can be seen in the corners of the mouth.
  6. The lips become inflamed, red and flaky.
  7. Iron deficiency leads to discoloration of the whites of the eye. The sclera acquires a bluish or bluish tint. The patient begins to see worse in poor lighting or at night. Eye diseases may occur: keratitis, conjunctivitis, blepharitis.
  8. Urine becomes reddish in color.
  9. Characteristic changes in the sense of smell and taste. The patient develops an addiction to unpleasant odors and spicy, salty or sour foods.
  10. The tongue becomes red and inflamed. Its surface is smoothed and looks varnished. This symptom can be found in 10% of patients with Plummer-Vinson syndrome. A photo of the patient's tongue can be seen below.

If you notice such symptoms, you should immediately consult a doctor. This pathology is treated by hematologists and gastroenterologists. The disease is dangerous with complications. Atrophic changes in the mucous membrane of the stomach and esophagus often lead to the formation of malignant tumors on these organs.

According to ICD-10, Plummer-Vinson syndrome is designated under code D50.1 as one of the types of iron deficiency anemia. Another name for this disease is sideropenic dysphagia.

Diagnostics

A preliminary diagnosis can be made based on the patient’s complaints and characteristic changes in the skin, nails, and tongue. This allows the doctor to suspect iron deficiency anemia in the patient. To clarify the nature of the pathology, it is necessary to examine the blood picture and the condition of the esophagus.

A detailed prescription is prescribed. In patients with Plummer-Vinson syndrome, reduced hemoglobin is detected, a change in the correct shape and size of red blood cells, and flattening of blood cells. In addition, you should be tested for ferritin levels. In patients, this indicator is usually sharply reduced.

An examination of the esophagus plays an important role in the diagnosis of Plummer-Vinson syndrome. It is necessary to take an x-ray of this organ, as well as an esophagoscopy. Patients experience thickening and keratinization of the esophageal epithelium. On x-rays you can see a “web” pattern in the upper part of the organ.

Treatment

Patients with Plummer-Vinson syndrome are prescribed iron supplements, medications containing riboflavin, folic acid, lactoflavin and pyridoxine. Patients need to take medications that improve gastric secretion and food digestion. A diet high in protein and vitamin B6 is recommended. It is useful for patients to include dairy products, liver, meat, eggs, and vegetables in their diet.

Forecast

In the early stages of Plummer-Vinson syndrome, timely treatment and diet will help achieve long-term remission and improved well-being. However, with severe symptoms, the prognosis of the disease is unfavorable. In advanced cases, it is almost impossible to cure this pathology.

Patients suffering from this syndrome should periodically undergo thorough gastroenterological and oncological examinations, since atrophic changes in the esophagus are considered a precancerous condition.

Prevention

Specific prevention of Plummer-Vinson syndrome has not currently been developed, since the exact causes of the pathology have not been established. However, you can reduce your chance of developing this disease. It is necessary to include foods rich in iron and B vitamins in the diet, lead an active lifestyle with reasonable physical activity and systematically monitor hemoglobin levels. All this will help minimize the risk of iron deficiency anemia.

Paterson-Kelly syndrome (D.R. Paterson, 1883-1939, English otorhinolaryngologist; A.V. Kelly, 1865-1941, Scottish otorhinolaryngologist)

1. Small medical encyclopedia. - M.: Medical encyclopedia. 1991-96 2. First aid. - M.: Great Russian Encyclopedia. 1994 3. Encyclopedic Dictionary of Medical Terms. - M.: Soviet Encyclopedia. - 1982-1984.

See what “Paterson-Kelly syndrome” is in other dictionaries:

    - (D. R. Paterson, 1883 1939, English otorhinolaryngologist; A. V. Kelly, 1865 1941, Scottish otorhinolaryngologist) see Plummer Vinson syndrome ... Large medical dictionary

    PATTERSON–KELLY SYNDROME- - same as Plummer-Vinson syndrome... Encyclopedic Dictionary of Psychology and Pedagogy

    - (H. S. PIummer, 1874 1937, American therapist; P. P. Vinson, American physician; synonym: Paterson Kelly syndrome, sideropenic syndrome) a disease presumably caused by a deficiency of riboflavin and folic acid and characterized by dysphagia, ... ... Large medical dictionary

    - (H.S. Plummer, 1874 1937, American physician; P.P. Vinson, American physician; synonym: Paterson Kelly syndrome, sideropenic syndrome) a disease presumably caused by a deficiency of riboflavin and folic acid and characterized by... ... Medical encyclopedia