Plummer–Vinson Syndrome

Plummer–Vinson Syndrome

Question. Classify disorders of red blood cells and discuss in detail Plummer-Vinson syndrome.
Or
Write a short note on Plummer-Vinson syndrome.
Or
Write a short note on the clinical features of Plummer-Vinson syndrome.
Answer.

Classification of Disorders of Red Blood Cells

Anemia

Plummer–Vinson syndrome symptoms

• Anemia of blood cells
• Acute
• Chronic
• Anemias of decreased red blood cell production
• Maturation defects
• Megaloblastic anemia
• Iron deficiency anemia
• Myelodysplastic syndromes
• Proliferation defects
• Stem cell defects
• Marrow replacement processes
• Anemia of chronic kidney disease
• Anemia of chronic disease
• Hemolytic anemias
• Intrinsic red cell defects
• Hemoglobin disorders
• Structural hemoglobin disorders
• Sickling diseases
• Hemoglobin C disease
• Thalassemias
• Red cell membrane disease
• Hereditary spherocytosis
• Hereditary elliptocytosis
• Hereditary pyropoikilocytosis
• Red cell enzyme disorders
• Glucose 6-phosphate dehydrogenase deficiency
• Paroxysmal nocturnal hemoglobinuria
• Extrinsic red cell defects
• Autoimmune hemolytic anemia
• Warm
• Cold
• Microangiopathic hemolytic anemia
• Parasitic hemolysis
• Malaria
• Babesia.

Iron deficiency anemia and dysphagia

Erythrocytosis

• Relative erythrocytosis
• Absolute erythrocytosis
• Primary
• Polycythemia vera
• Primary familial polycythemia
• Secondary
• Chronic hypoxia
• Cardiac disorders
• Pulmonary disorders
• High altitude residence
• Hemoglobin abnormalities
• High oxygen-affinity hemoglobins
• Increased carboxyhemoglobins
• Aberrant erythropoietin is produced by tumors.

Plummer-Vinson Syndrome

It is also known as Paterson-Brown-Kelly Syndrome.

This syndrome consists of the following i.e., glossitis, iron deficiency anemia, koilonychia, and dysphagia.

Plummer-Vinson Syndrome Clinical Features

• The syndrome is seen in middle-aged people, and a female predilection is seen.
• Individuals suffering from the syndrome have a sthenic appearance.
• There is a presence of difficulty in swallowing, i.e., dysphagia, because ofthe formation of webs in the esophagus.
• Angular cheilitis is present, i.e., cracking ofthe corners of the lip.
• Tongue becomes red in colour and smooth. At times, tongue get enlarged and there is sore tongue along with the fissuring.
• The oral cavity appears to be pale.
• Presence of xerostomia.
• Koilonychia is present, i.e., spoon-shaped nails are seen. The width of the mouth is narrowed, and the oral mucosa is pale and painful.

Plummer-Vinson Syndrome Diagnosis

• Clinical diagnosis: The Presence of dysphagia, signs and symptoms of iron deficiency anemia, and koilonychia confirms the diagnosis.
• Laboratory diagnosis: Presence of features of iron deficiency anemia. In biopsies, atrophy of epithelium, lamina propria, and muscles is evident.

Risk of esophageal cancer in Plummer–Vinson syndrome

Plummer-Vinson Syndrome Management

• Give an iron supplement for treating iron deficiency anemia.
• Esophageal dilatation is done in cases with long-standing dysphagia. Needle knife electroincision as an alternative can also be carried out.
• Disruption of esophageal webs is carried out by endoscope/ Nd: YAG laser therapy.