What is the management plan for Paterson-Kelly syndrome?

Management of Paterson-Kelly Syndrome

The management of Paterson-Kelly syndrome should focus primarily on iron supplementation to correct the underlying iron deficiency anemia, along with endoscopic treatment of esophageal webs when dysphagia is present. 1

Diagnosis and Evaluation

• Paterson-Kelly syndrome (also known as Plummer-Vinson syndrome) is characterized by iron deficiency anemia, dysphagia, and esophageal webs 1
• The syndrome is associated with iron deficiency anemia through the Paterson-Brown-Kelly syndrome mechanism 1
• Comprehensive evaluation should include:
  • Assessment for iron deficiency anemia with serum ferritin as the preferred initial diagnostic test 2
  • Endoscopic evaluation to identify esophageal webs and rule out malignancy 1
  • Investigation for underlying causes of iron deficiency, including celiac disease which has been reported as an etiological factor for sideropenic dysphagia 3

Treatment Approach

Iron Supplementation

• Oral iron therapy is the first-line treatment for correcting the underlying iron deficiency anemia 4, 5
• Recommended dosing:
  • For symptomatic patients or those with severe anemia: 200 mg ferrous fumarate daily 6
  • For asymptomatic patients with mild anemia: 100 mg ferrous fumarate once daily may be sufficient 6
  • If side effects occur, consider alternate-day dosing to improve tolerance 6
• Monitor hemoglobin response after one month of therapy; expect a 1-2 g/dL increase if treatment is effective 2

Parenteral Iron Therapy

• Consider intravenous iron supplementation in cases where:
  • Oral iron is not tolerated due to side effects 5
  • Gastrointestinal blood loss exceeds intestinal ability to absorb iron 4
  • Rapid correction of anemia is needed 6
  • Patient has failed to respond to oral iron therapy 5

Endoscopic Management

• Endoscopic dilation of esophageal webs should be performed in patients with significant dysphagia 1
• The procedure typically provides immediate relief of dysphagia symptoms 1
• Multiple dilations may be necessary in some cases 1

Long-term Management and Follow-up

• Regular monitoring of iron status and hemoglobin levels to ensure maintenance of normal iron stores 5
• Surveillance endoscopy should be considered due to the increased risk of developing squamous cell carcinoma of the esophagus in these patients 1
• Nutritional counseling to encourage consumption of iron-rich foods and vitamin C to enhance iron absorption 1

Special Considerations

• Investigate for celiac disease in patients with Paterson-Kelly syndrome, as it can be an underlying cause of chronic iron deficiency leading to this syndrome 3
• In patients over 50 years of age with iron deficiency anemia, colonoscopy should be performed to rule out gastrointestinal malignancy 2
• Women of reproductive age may benefit from a trial of iron supplementation if review of symptoms and physical examination are negative 2

Common Pitfalls to Avoid

• Failing to investigate the underlying cause of iron deficiency anemia, which could lead to missed diagnoses of conditions like celiac disease or gastrointestinal malignancies 3, 2
• Discontinuing iron therapy prematurely before iron stores are fully replenished 4
• Overlooking the need for endoscopic dilation in patients with significant dysphagia 1
• Using inadequate iron dosing or not addressing adherence issues with oral iron therapy 6