Plummer-Vinson syndrome is understood by medicine to be a triad of dysphagia, iron deficiency, and atrophy of the esophagus that occurs as a result of long-standing iron deficiency. Therapy is causal, in that the iron deficiency is compensated and the symptoms thus regress. The untreated syndrome promotes carcinoma.
What is Plummer-Vinson syndrome?
Plummer-Vinson syndrome is a symptom complex resulting from long-standing iron deficiency. The syndrome is also known as sideropenic dysphagia or Paterson-Brown-Kelly syndrome. The disease is named after the U.S. internist H. S. Plummer and the surgeon P. Vinson, who first described the symptom complex in the 20th century. Characteristic of Plummer-Vinson syndrome are mainly trophic disturbances. As such, nutritional disturbances in the innervation area of nerves are known. In addition to discoloration of the skin, a disturbance of thermoregulation sometimes occurs in the context of trophic disturbance. The syndrome is broadly classified as anemia, since iron deficiency anemia underlies the symptoms. Accurate data on the prevalence of the disorder do not exist, but the syndrome is thought to be rare. Women of European descent in the fourth to seventh decades of life are most commonly affected. Nonetheless, the disease has also been observed in children and adolescents.
Causes
The cause of Plummer-Vinson syndrome is iron deficiency anemia that persists over a long period of time. In long-term and severe iron deficiency, atrophies form on the mucous membranes, often accompanied by submucosal inflammatory reactions. These atrophies predominantly affect the mucous membranes of the oral cavity and esophagus in the context of iron deficiency in the sense of Plummer-Vinson syndrome. In the esophagus, impaired growth and progressive regeneration cause membranes to develop that cause swallowing difficulties and make swallowing painful, for example. Many other factors must be considered as primary causes of iron deficiency. In addition to malnutrition, for example, genetic predisposition may be involved in the occurrence of the syndrome. The same applies to autoimmune processes. The relevance of these additional factors is the current subject of research and, consequently, has not yet been conclusively confirmed.
Symptoms, complaints, and signs
Plummer-Vinson syndrome usually consists of a triad: in addition to dysphagia, iron-deficiency anemia and esophageal atrophy are present. Dysphagia may or may not be associated with pain. Often, dysphagia increases with years of its persistence. Because it primarily affects solid food, it often evokes weight loss. Plummer-Vinson syndrome patients also suffer from the typical symptoms of anemia. Besides weakness, these symptoms include striking pallor, easy fatigability, and sometimes tachycardia. Additionally, some patients suffer from glossitis, cheilitis in the corners of the mouth, or nail changes such as koilonychia. Both the spleen and thyroid gland of patients may be enlarged. Splenomegaly is an equally conceivable symptom. If left untreated, Plummer-Vinson syndrome can cause atypia or dysplasia of the upper esophageal epithelium and therefore must be understood as a risk factor for cancers such as esophageal carcinoma or squamous cell carcinoma. The development of carcinoma from Plummer-Vinson syndrome usually takes time and can be prevented by timely treatment in most cases.
Diagnosis and disease progression
Diagnosis for Plummer-Vinson syndrome is made by history and blood work. The blood count reveals iron deficiency anemia with a typical hypochromic microcytic aspect. Iron deficiency can be confirmed by laboratory determination of serum iron or ferritin. To confirm the suspected diagnosis of Plummer-Vinson syndrome, the treating physician can detect the pathologically typical changes in the mucous membranes with the aid of gastroscopy of the esophageal membranes. Histopathological tissue changes are investigated by a biopsy and thus the removal and examination of mucosal tissue. A biopsy can also confirm or rule out the suspicion of pre-existing carcinoma of the upper esophageal tract. The prognosis for Plummer-Vinson syndrome treated early is favorable. However, if left untreated, the risk for carcinoma is high and the prognosis is correspondingly unfavorable.
Complications
Due to Plummer-Vinson syndrome, affected individuals primarily suffer from anemia. As a result, the patient’s ability to work under pressure is significantly reduced and, furthermore, the patient suffers from persistent fatigue. The affected persons also appear pale and no longer actively participate in everyday life. Weight loss can also occur as a result of Plummer-Vinson syndrome and is usually associated with other deficiency symptoms. Plummer-Vinson syndrome increases the risk of various cancers, so sufferers are usually dependent on various therapies and screenings. Likewise, Plummer-Vinson syndrome can lead to psychological discomfort or upset, with depression occurring in the worst cases. The patient’s quality of life is significantly reduced and limited by this disease. As a rule, the diagnosis is made relatively quickly, so that early treatment of Plummer-Vinson syndrome is also possible. The treatment is carried out with the help of medication or through various interventions. Complications usually do not occur and the disease always progresses positively. With early diagnosis and treatment of Plummer-Vinson syndrome, the patient’s life expectancy is usually not reduced.
When should you see a doctor?
Plummer-Vinson syndrome must be treated by a doctor in all cases, as this does not result in self-healing and the symptoms usually continue to worsen. Since Plummer-Vinson syndrome also favors the occurrence of tumors, patients are dependent on regular examinations in order not to reduce their life expectancy. The doctor should be consulted in the case of this syndrome if the affected person suffers from swallowing difficulties, which can occur at a young age. Also, sudden weight loss may indicate the syndrome and must be examined by a physician. The corners of the patient’s mouth are often torn open and there is severe pallor, fatigue and listlessness. In many cases, discoloration of the nails also indicates Plummer-Vinson syndrome and should be examined by a physician. The diagnosis and treatment of this disease is carried out by various specialists and always depends on the exact manifestation of the disease. In most cases, this results in a reduced life expectancy.
Treatment and therapy
Causal treatment is the primary focus of therapy in Plummer-Vinson syndrome. The causative iron deficiency must be treated and, at best, compensated. Oral administration of iron supplements is usually ordered to treat the iron deficiency, which may extend over several months depending on the severity of the syndrome. In exceptional cases, the administration of the preparations can also be done parental. Once the patient’s causative anemia improves, the symptoms of Plummer-Vinson syndrome usually resolve on their own. If iron supplementation is not sufficient to remedy the symptoms, mechanical dilatation of the affected esophageal areas may be performed. However, Plummer-Vinson syndrome is usually already effectively treated by the preparations. However, if the lumen of the esophagus has significantly shifted through the mesh, dysphagia persists and further corrective measures must follow. As such, rupture of the esophageal mesh may be considered. After completion of treatment, close monitoring of patients is recommended. Indeed, even after resolution of symptoms, Plummer-Vinson syndrome patients are at increased risk of squamous cell carcinoma of the pharynx or esophagus. Surveillance should ideally occur at three-month intervals to detect any cancer cells early enough.
Prevention
Plummer-Vinson syndrome can be prevented. Because iron deficiency anemia is the cause of the condition, according to current research, a balanced diet can help prevent it. If iron deficiency does not develop in the first place, the symptom complex will not occur.
Follow-up
In most cases, the person affected by Plummer-Vinson syndrome has very few or no special measures or options for aftercare. The primary focus is on rapid and, above all, early detection of the disease to prevent further worsening of symptoms or other complications.The affected person should therefore contact a doctor at the first signs of Plummer-Vinson syndrome and initiate treatment. Most of those affected depend on taking various medications and supplements in order to alleviate the symptoms permanently and, above all, correctly. Regular intake and correct dosage of the medication should always be observed to avoid further complications. Since Plummer-Vinson syndrome also significantly increases the likelihood of tumors, those affected should have themselves checked regularly by a doctor. This can also prevent further spread of the tumor. Often, those affected are also dependent on the help and support of their own family, which can also prevent psychological upsets or depression. In this context, loving and intensive conversations with one’s own family are often very important.
This is what you can do yourself
Since the cause of Plummer-Vinson syndrome is due to iron deficiency anemia that has persisted over a long period of time, the attending physician will prescribe an iron-containing preparation. However, since iron is mainly absorbed through food, its absorption can also be supported at home. Foods with a high iron content are recommended for this purpose. A particularly high concentration of iron is present in animal products, preferably in red meat such as beef. Somewhat less is present in chicken and turkey meat. Plant sources include dark varieties of lettuce, for example arugula and lamb’s lettuce. Grains such as oats, millet and buckwheat also contain iron. In addition to these foods on the menu, however, it is also important to note that certain components inhibit or facilitate iron absorption. For example, you should always make sure to take vitamin C before or during meals, for example in the form of orange juice or fresh fruit. Since caffeine and calcium inhibit the absorption of iron in the body, a distance of one to two hours should be observed from the consumption of coffee, black tea as well as dairy products. Iron absorption through food can therefore be supported. However, treatment with a dietary supplement prescribed by a physician should not be replaced by diet in Plummer-Vinson syndrome.