What is the appropriate management for a patient with severe iron deficiency anemia, buccal and oral pigmentation, and goitre?

Management of Severe Iron Deficiency Anemia with Buccal/Oral Pigmentation and Goitre

Oral iron supplementation with ferrous sulfate 200 mg three times daily is the first-line treatment for severe iron deficiency anemia with buccal/oral pigmentation and goitre, while investigating and treating the underlying cause. 1

Diagnostic Evaluation

• Evaluate ferritin levels with a cut-off value of 45 mg/dL for diagnosing iron deficiency anemia, considering that inflammatory conditions may have higher ferritin levels (usually <100 mg/dL) 1
• Assess transferrin saturation, soluble transferrin receptor, or reticulocyte hemoglobin equivalent as confirmatory tests when ferritin results are ambiguous 1
• Investigate for thyroid dysfunction given the presence of goitre, which may be contributing to or coexisting with iron deficiency anemia 2
• Screen for celiac disease with antiendomysial antibodies and IgA measurement, as it is present in 2-6% of asymptomatic patients with iron deficiency anemia 1
• Evaluate for potential gastrointestinal sources of blood loss with both upper GI endoscopy with small bowel biopsy and colonoscopy or barium enema 1

Treatment Approach

Iron Supplementation

• Begin with oral ferrous sulfate 200 mg three times daily (providing approximately 65 mg elemental iron per tablet) 1, 3
• Consider alternative oral formulations like ferrous gluconate or ferrous fumarate if ferrous sulfate is not tolerated 1
• Add ascorbic acid (vitamin C) to enhance iron absorption, particularly when response is poor 1, 2
• Administer oral iron on an empty stomach when possible to maximize absorption 1
• Expect hemoglobin concentration to rise by approximately 2 g/dL after 3-4 weeks of treatment 1, 2

Management of Goitre

• Evaluate thyroid function tests to determine if hypothyroidism is present, which can exacerbate anemia 2
• Treat any identified thyroid dysfunction appropriately alongside iron supplementation 2

When to Consider Intravenous Iron

• Switch to intravenous iron if there is intolerance to at least two oral preparations, non-compliance, or inadequate response to oral therapy 1, 2
• Consider intravenous iron initially in cases of severe iron deficiency anemia (Hb <10 g/dL) requiring rapid correction 2, 4
• Use intravenous iron formulations that can replace iron deficits with 1-2 infusions 1, 2
• Be aware that true anaphylaxis with intravenous iron is rare; most reactions are infusion-related 1, 2

Monitoring and Follow-up

• Recheck hemoglobin and iron studies after 3-4 weeks of treatment to assess response 1, 2
• Continue treatment until anemia is corrected and iron stores are replenished 2
• Investigate further if anemia doesn't resolve within 6 months 1, 2
• Consider intermittent oral iron supplementation for patients with repeatedly low ferritin to maintain iron stores 5

Special Considerations for Buccal/Oral Pigmentation

• Buccal and oral pigmentation may be a manifestation of Plummer-Vinson syndrome (also known as Paterson-Kelly syndrome), which is associated with iron deficiency anemia and requires thorough evaluation 6, 7
• Monitor for improvement in oral pigmentation with iron repletion, as this can be a clinical marker of treatment response 7

Common Pitfalls to Avoid

• Failing to investigate the underlying cause of iron deficiency anemia while treating the anemia 2, 8
• Continuing oral iron therapy despite poor response or intolerance 2, 4
• Not considering potential malabsorption issues, especially with concurrent thyroid disease 1, 2
• Overlooking the need for multidisciplinary management involving gastroenterologists, hematologists, and endocrinologists in complex cases 1, 2