The picture of his blood film is shown below:
What is the Diagnosis:
Sickle Cell Anemia
Case Discussion:
Sickle cell anemia is an autosomal recessive disease resulting from the substitution of valine for glutamic acid at sixth position in the globin chain.
Clinical signs And Symptoms:
Sickle cell patients often have a positive family history. they may also have a history of gall stones and poorly healing ulcers. On examination they may have significant jaundice, splenomegaly (in childhood) or even congestive heart failure when anemia is severe.
Diagnosis: The definitive diagnosis is done by hemoglobin electrophoresis. The blood film may show sickle shaped cells.
Treatment:
1. Patients should be instructed to avoid dehydration, hypoxia, intense exercise and high altitudes.
2. Patients should be given folic acid supplements.
3. Vaccination for streptococcus pneumoniae, hemophilus influenzae and hepatitis B is recommended.
4. Transfusion may be needed when anemia is severe.
Complications:
1. Pain crisis: It is characterized by pain in the back, limbs and ribs. It is caused by tissue infarction when the sickled cells occlude the arterioles. It is precipitated by dehydration, acidosis, fever or hypoxia.
2. Aplastic Crisis: A sudden decrease in hemoglobin and reticulocyte count caused by parvovirus B19 infection. Supported by blood transfusions.
3. Acute chest crisis: Sickled cells can cause occlusion of pulmonary blood supply leading to lung infarctions. The clinical findings include a fever, chest pain, cough , wheezing, tachypnea and a new pulmonary infiltrate on chest x ray.
4. Infections: Patients are predisposed to Streptococcus pneumoniae, hemophillus influenzae, Neisseria meningitidis and gram negative bacterial infections.
5. Bones: Patients may suffer from avascualr necrosis or Salmonella osteomyelitis.
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