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Ochsner Journal 2019Plummer-Vinson syndrome (PVS), a rare disorder characterized by dysphagia, iron deficiency anemia, and esophageal webs, has principally been described in middle-aged...
Plummer-Vinson syndrome (PVS), a rare disorder characterized by dysphagia, iron deficiency anemia, and esophageal webs, has principally been described in middle-aged women. This disorder is uncommon in the 21 century because of the abundance of iron-fortified foods. Clotting factor deficiencies are also rare. Factor VII deficiency is a bleeding disorder characterized by the absence of a critical protein in the coagulation cascade. We present a case of PVS associated with factor VII deficiency in a 26-year-old African American female. The patient had a history of anemia that was repeatedly attributed to menstrual bleeding and dysphagia for 10 years. She presented with symptomatic anemia requiring transfusion. She reported a history of food getting stuck in her chest, and workup revealed esophageal webs with no evidence of overt luminal gastrointestinal bleeding. Coagulation laboratory tests revealed the incidental finding of a borderline increased prothrombin time. Hematologic studies confirmed the presence of factor VII deficiency. To our knowledge, no case has been published about a patient diagnosed with PVS and concomitant factor VII deficiency. Our case illustrates several learning points: (1) PVS is an uncommon disorder that may still be diagnosed in a developed country in the 21 century; (2) PVS requires close follow-up and esophageal surveillance because of the increased risk of esophageal cancer; (3) factor VII exhibits a high degree of phenotypic variability; (4) phenotype in factor VII deficiency does not always correlate with factor VII activity, although life-threatening spontaneous bleeding is not expected with levels >2%.
PubMed: 31528144
DOI: 10.31486/toj.18.0158 -
The American Journal of Case Reports Aug 2019BACKGROUND Plummer-Vinson syndrome (PVS) is a rare disorder composed of the triad of dysphagia, iron-deficiency anemia (IDA), and esophageal webs. It is most prevalent...
BACKGROUND Plummer-Vinson syndrome (PVS) is a rare disorder composed of the triad of dysphagia, iron-deficiency anemia (IDA), and esophageal webs. It is most prevalent in middle-aged white women, and the dysphagia often improves when the anemia is treated. It is well established that chronic hypertension can lead to congestive heart failure (CHF). While IDA is frequently found concomitantly with CHF, there have been no reported cases of new-onset CHF with anemia presenting as PVS. CASE REPORT We present the case of a 48-year-old African American woman with symptomatic anemia and new-onset congestive heart failure secondary to hypertension, who presented with the classic symptoms of PVS. CONCLUSIONS CHF with accompanying IDA may be an independent risk factor for the development of PVS. At the very least, there is an association between CHF-induced IDA and PVS. Patients presenting with CHF with symptoms of dysphagia should be considered at risk for the syndrome, and endoscopy may be warranted. Treatment for PVS includes iron replacement, and in some cases requires mechanical dilation.
Topics: Black or African American; Female; Heart Failure; Humans; Hypertension; Middle Aged; Plummer-Vinson Syndrome
PubMed: 31451679
DOI: 10.12659/AJCR.916823 -
Journal of Multidisciplinary Healthcare 2019Plummer-Vinson syndrome is a rare condition associated with dysphagia, iron deficiency, and esophageal webs. Data regarding this condition is limited to mostly case... (Review)
Review
Plummer-Vinson syndrome is a rare condition associated with dysphagia, iron deficiency, and esophageal webs. Data regarding this condition is limited to mostly case reports and a few small cohort studies. Although most cases have a benign and indolent course, the risk of malignancy warrants long-term surveillance. A multidisciplinary approach among healthcare providers is of the utmost importance in the management of this condition.
PubMed: 31417270
DOI: 10.2147/JMDH.S180410 -
Ear, Nose, & Throat Journal Jun 2019
Topics: Anemia, Iron-Deficiency; Dyspnea; Fatigue; Female; Humans; Middle Aged; Plummer-Vinson Syndrome
PubMed: 31208220
DOI: 10.1177/0145561319850412 -
Internal Medicine (Tokyo, Japan) Mar 2019Plummer-Vinson syndrome is a rare entity, characterized by dysphagia, esophageal web formation, and iron deficiency anemia. The patient was a 63-year-old woman with a...
Plummer-Vinson syndrome is a rare entity, characterized by dysphagia, esophageal web formation, and iron deficiency anemia. The patient was a 63-year-old woman with a clinical history of iron deficiency anemia and glossitis in her 20s to 40s and who had experienced swallowing difficulties for the past 20 years. A membranous stricture was found in the cervical esophagus during a fluoroscopic examination. An endoscopic examination conducted under general anesthesia revealed an oblique linear scar on the proximal surface of the stricture. Sequential balloon dilation was performed successfully. We suggest that the esophageal web formation might have been related to the healing of an esophageal ulcer.
Topics: Esophagoscopy; Esophagus; Female; Humans; Middle Aged; Plummer-Vinson Syndrome
PubMed: 30449792
DOI: 10.2169/internalmedicine.1628-18 -
The Permanente Journal 2018
Topics: Deglutition Disorders; Esophageal Stenosis; Esophagoscopy; Esophagus; Female; Humans; Middle Aged; Plummer-Vinson Syndrome; Radiography
PubMed: 30005727
DOI: 10.7812/TPP/18-035 -
Anales de Pediatria May 2019
Topics: Anemia, Iron-Deficiency; Child; Deglutition Disorders; Female; Humans; Plummer-Vinson Syndrome
PubMed: 29773525
DOI: 10.1016/j.anpedi.2018.03.019 -
Blood Research Mar 2018
PubMed: 29662867
DOI: 10.5045/br.2018.53.1.79 -
Przeglad Gastroenterologiczny 2017
PubMed: 29123586
DOI: 10.5114/pg.2017.70477 -
Journal of Blood Medicine 2017Plummer-Vinson syndrome (PVS), a rare clinical condition, is characterized by a triad of dysphagia, iron deficiency anemia and esophageal web in the post-cricoid region.... (Review)
Review
Plummer-Vinson syndrome (PVS), a rare clinical condition, is characterized by a triad of dysphagia, iron deficiency anemia and esophageal web in the post-cricoid region. It was first described over a century ago. However, literature on this condition remains scanty, and its prevalence appears to be declining worldwide, possibly due to improvements in nutrition over time. The condition has been reported most commonly in thin-built, middle-aged, white women. The esophageal webs in PVS are thin mucosal folds, which are best seen either in lateral views at barium swallow or at esophagoscopy. These are usually semilunar or crescentic, being located most often along the anterior esophageal wall, but can be concentric. The exact cause and pathogenesis of PVS remain unclear, though iron and other nutritional deficiencies, genetic predisposition and autoimmunity have all been implicated in formation of the webs. Treatment includes correction of iron deficiency and endoscopic dilation of the esophageal webs to relieve dysphagia. PVS is associated with an increased risk of hypopharyngeal and esophageal malignancies. Correction of iron deficiency may arrest and reverse the mucosal changes and possibly reduces this risk.
PubMed: 29089792
DOI: 10.2147/JBM.S127801