Plummer-Vinson Syndrome and Iron Deficiency Anemia
Direct Answer
Plummer-Vinson syndrome (PVS) is defined by the classic triad of iron deficiency anemia, post-cricoid esophageal webs, and dysphagia, occurring predominantly in middle-aged women (85.6% female, mean age 43.5 years). 1
Clinical Features and Demographics
Key Demographics:
- Affects primarily middle-aged women (85.6% female) with mean age of 43.5 years 1
- Now rare in developed countries due to improved nutritional status and early iron deficiency treatment 2
Classic Triad:
- Iron deficiency anemia (microcytic, hypochromic) 3, 4
- Post-cricoid esophageal webs causing upper esophageal obstruction 1, 5
- Dysphagia (typically early grade, progressive difficulty swallowing solids) 1, 4
Additional Oral and Mucosal Manifestations:
- Angular cheilitis (inflammation at mouth corners) 3, 4
- Atrophic glossitis (smooth, red tongue) 3, 4
- Koilonychia (spoon-shaped nails) 3, 5
- These features reflect chronic iron deficiency and help distinguish PVS from isolated esophageal pathology 6
Pathophysiology
The esophageal webs in PVS are directly caused by chronic severe iron deficiency, though the exact mechanism remains incompletely understood. 3 The webs typically form in the post-cricoid region of the upper esophagus and consist of thin mucosal membranes that partially obstruct the lumen. 1, 5
Diagnostic Approach
Laboratory Confirmation:
- Serum ferritin <15 μg/L confirms iron deficiency 7
- Complete blood count showing microcytic hypochromic anemia 3, 4
- Low iron saturation and elevated total iron-binding capacity 8
Endoscopic Evaluation:
- Upper endoscopy reveals post-cricoid web preventing endoscope passage 1, 3
- Barium-swallow esophagography demonstrates web as thin filling defect in upper esophagus 3, 5
- Duodenal biopsies must be obtained during upper endoscopy to screen for celiac disease (found in 3-5% of iron deficiency cases) 6, 7
Mandatory Additional Investigation:
- Colonoscopy to exclude lower GI blood loss sources (colorectal cancer, polyps) 6, 8
- Urinalysis to exclude urinary tract bleeding 7, 9
- Celiac disease serologic screening with tissue transglutaminase antibody 6, 7
Management Algorithm
Step 1: Iron Replacement Therapy
- Oral ferrous sulfate 324 mg (65 mg elemental iron) three times daily on empty stomach 8
- Add vitamin C 500 mg with each dose to enhance absorption 8
- Continue for 3 months after hemoglobin normalizes to replenish body stores 8
Step 2: Mechanical Dilation
- Single session Savary-Gilliard bougie dilation is successful in 90.7% of patients for relieving dysphagia 1
- Alternative: balloon dilation can be used 5
- Only 9.3% develop recurrence requiring repeated dilations 1
Critical Clinical Pearl:
- Dysphagia and esophageal webs can improve dramatically within 2 weeks of iron therapy alone, sometimes before mechanical dilation 3
- However, most patients still require dilation for complete symptom resolution 1, 5
Malignancy Risk and Surveillance
PVS carries a 4.5% overall risk of developing upper gastrointestinal malignancy, specifically squamous cell carcinoma of the esophagus or pharynx. 1, 4 This represents a critical long-term complication requiring ongoing surveillance.
Surveillance Protocol:
- Monitor CBC and mean cell volume every 3 months for 1 year, then at 2 years 8
- Recheck ferritin after 3 months of iron therapy (target >50 ng/mL) 8
- Maintain clinical vigilance for recurrent dysphagia or new upper GI symptoms suggesting malignant transformation 1, 4
Common Pitfalls to Avoid
Do not assume iron deficiency alone explains the presentation without investigating underlying causes:
- Screen for celiac disease in all cases (3-5% prevalence) 6, 7
- Explicitly ask about NSAID use (including over-the-counter ibuprofen, naproxen, aspirin) as this causes occult GI blood loss 9, 8
- Complete bidirectional endoscopy even after finding the esophageal web, as dual pathology occurs in 1-10% of cases 6, 9
Do not stop investigation after treating the web: