Patient with Dyspnea, Dysphagia and Menorrhagia: Plummer — Vinson Syndrome Against the Background of von Willebrand Disease

Aim: to present a clinical observation of a patient with Plummer — Vinson syndrome against the background of von Willebrand disease.
Key points. A 40-years-old woman presented to the hematology department of our hospital with fatigue, dizziness, dyspnea on exertion and dysphagia to solid food. Laboratory examination showed microcytic and hypochromic anemia (haemoglobin — 2,0 g/dL; red blood cells — 1.31 × 1012/L), a reduction in serum levels of ferritin to 1.5 ng/mL and a decrease in the von Willebrand factor activity up to 32.4 %. According to esophagogastroduodenoscopy the lumen of the upper esophagus third was circularly narrowed to 8 mm by web formation. Combining these clinical symptoms and examinations, we made the diagnosis of von Willebrand disease and Plummer — Vinson syndrome. Iron supplementation therapy and replacement therapy with von Willebrand factor concentrate were prescribed. At the time of discharge, the patient noted a significant improvement in her general condition, the number of red blood cells was 3.9 × 1012/L, haemoglobin — 8.6 g/L.
Conclusion. Plummer — Vinson syndrome is a rare manifestation of iron deficiency anaemia and therefore can often be overlooked by physicians. The cause of deep tissue iron deficiency in our patient was chronic blood loss against the background of von Willebrand disease. Our literature review did not reveal similar cases of concomitant von Willebrand disease with Plummer — Vinson syndrome.

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