Sickle Cell Anemia

Sickle Cell Anemia

Question. Describe the etiopathogenesis, clinical manifestations, investigations, radiographic features, and dental considerations for sickle cell anemia.
Answer. Sickle cell anemia is a chronic hemolytic disorder of blood which is characterized by deoxygenated hemoglobin which, during decreased oxygen tension leads to sickling of cell. It is an inherited disorder.

Etiopathogenesis

Sickle cell anemia is an autosomal recessive type of inherited disease, which is characterized by a point mutation in the Hb gene, which results in an abnormal Hb molecule (HbS). When HbS is deoxygenated it forms tactoids. Each person inherits one gene from each parent, which governs the synthesis of hemoglobin. Tactoids will distort RBC membrane and lead the RBC into sickle shape. This crescent shaped or sickle- shaped RBC cells in sickle cell anemia are more prone to agglutination. More agglutinations (hemolysis) of RBC leads to more anemia and increased viscosity of blood, moreover increased risk of blocking of capillaries may result in ischemic damage in various organs and the situation is known as ’sickle cell crisis’.

Sickle cell anemia etiology

Etiopathogenesis Clinical Manifestations

• Disease is seen more commonly in females.
• Delayed physical growth and development of the patient.
• Malaise, weakness, and jaundice with a yellow sclera.
• Pallor, loss of appetite, dehydration and muscle rigidity
• Loss of consciousness is a common feature in severe cases.
• Children do not develop any symptom until late in the first year of life.
• Extreme susceptibility to infections, renal failure and CNS disturbances are common.
• There can be death due to widespread ischemia, hypoxia and hypothermia.
• Extreme pain in abdomen, lung, long bones and joints due to ischemia and infarction.
• Fever, swelling of the joints, hands and feet, etc. are also common.

Sickle cell crisis symptoms

Etiopathogenesis Investigations

• Blood tests:
  • RBC count may reach at the level of 1 lakh cells or less per cubic mL of blood.
  • Hemoglobin get decreased and ranges from 5g/dL to 12 g/dL
  • Reticulocyte count is relatively high
• Blood picture
  • On blood smear typical sickle shaped RBCs are commonly seen
• Levels of lactate dehydrogenase get increased and levels of hepatoglobin get decreased.
• Hemoglobin electrophoresis is done to differentiate homozygous with heterozygous.

Etiopathogenesis Radiographic Features

• Due to increased erythropoietic activity and marrow hyperplasia, trabeculae of jaw bone lost which causes mild to severe generalized osteoporosis and appearance of large irregular marrow spaces.
• Since radiolucency of jaw is increased, it produces ground glass appearance which is due to diminish of fine trabeculae as there is replacement of the marrow by compensatory hyperplasia.
• Inferior border of mandible get thinned.

Anemia and mandibular changes

• Lamina dura appears to be prominent.
• Trabeculae present between the roots of teeth appear as horizontal rows which produces step ladder like effect.
• Maxilla appears to be enlarged along with protrusion and separation of maxillary anterior teeth.
• Hair on end appearance should be appreciated on the skull radiographs. It is due to the perpendicular trabeculation which radiate outward from the inner table. Thickening of frontal and orbital bones is evident. Outer table of bone is seen to be absent and dipoles get thickened.

Step ladder trabeculae in jaw

Etiopathogenesis Dental Considerations

• Maintain proper asepsis for patients and use sterilized instruments.
• Dental procedures should be of short duration for the sickle cell anemia patients.
• General anesthesia should be avoided in cases sickle cell anemia. This is done to avoid the episodes of hypoxia because of cerebral and myocardial thrombosis which can occur.
• Dentition should be as healthy as possible since there are chances of infection which can precipitate aplastic crisis.