Sunday 18 August 2019

Sickle Cell Anemia MRI

Multiple vertebrae show subchondral collapse in dorso lumbar region.
Hip screening show avascular necrosis involving bilateral capital femoral epiphysis..
Ill-defined heterogeneous marrow oedema involving Acetabular fossa, Femoral head neck on either side, mild joint effusion.

Suggested detailed haematological / bone marrow evaluation.
On further evaluation, turned out to be a case of sickle cell anaemia.

Sickle cell disease

A hereditary autosomal recessive condition resulting in the formation of abnormal hemoglobin which manifests as multisystem ischemia and infarction as well as hemolytic anemia. 
There is no gender predilection. 
Highest incidence occurs in individuals of African descent, followed by eastern Mediterranean and Middle Eastern populations. 
The sickle cell mutation is prevalent in part as it confers a human genetic resistance to malaria as the abnormal hemoglobin has higher turnover and increased phagocytosis while sickled red cells have reduced cell-cell cytoadherence preventing the parasite from multiplying during the erythrocytic phase of its life cycle. It is estimated that approximately 8% of the African population is homozygous for sickle cell where malaria is most prevalent.
The earliest manifestation is usually in early childhood, as babies are protected by elevated levels of fetal hemoglobin (HbF) in the first 6 months3. 
Commonly a painful vaso-occlusive crisis, sudden onset of bone or visceral pain due to microvascular occlusion and ischemia.
Three separate mechanisms can result in skeletal changes: 
1. chronic anemia resulting in expansion of the medullary spaces
2. vaso-occlusive crises resulting in bone infarcts and subperiosteal hemorrhages

3. infection, osteomyelitis.

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