Thursday, April 23, 2015

7 Year Old Male Child Presents With Pallor and Dark Urine While On Treatment For Malaria

A 7 Year old child who presented with high grade fever about 5 days back and is now on treatment for malaria now presents to the emergency with severe, sudden onset of pallor and dark colored urine. He feels very weak and is frightened by the appearance of his urine. Although he is afebrile now, he is breathing rapidly and on examination he looks pale and there is yellowish discoloration of his skin and sclera. On abdominal examination, spleen is enlarged and palpable.

Labwork shows:
Hb = 5 gm/dl
Increased reticulocyte count
Increased billirubin levels.

The peripheral blood film has the following picture:

Numerous destroyed red blood cells ( Arrows shows bite cells following removal of Heinz bodies).


What is the Diagnosis?
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.
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Hemolytic anemia due to G6PD deficiency, in this case hemolysis triggered due to antimalarial drugs.


Case Discussion:
Glucose 6 Phosphate dehydrogenase deficiency anemia is due to lack of an important enzyme glucose 6 phosphate dehydrogenase and the condition predisposes to spontaneous destruction of red blood cells and hemolysis due to exposure to certain food and medicines.
It is an X linked inherited disorder and most commonly seen in males.

Causes: Following triggers may lead to hemolysis:

  • Infections
  • Stress
  • Ingestion of fava beans
  • Certain antimalarial drugs
  • Sulfa drugs
  • Aspirin
  • Exposure to certain chemicals.
Clinical Features: Patients are usually asymptomatic until an exposure to the susceptible trigger. They may give recent history of an infection or use of certain drugs. Patients may present with:
  • Pallor
  • Fatigue
  • Dark urine
  • Jaundice
  • Enlarged spleen
  • Fever
  • Shortness of breath
  • Rapid heart beat
Management: For diagnosis a complete blood count with reticulocyte count and a peripheral blood film is helpful. Also Billirubin levels are high because of underlying hemolysis. Treatment involves stopping the drug responsible for hemolysis, and treating the underlying infection. In severe cases of anemia blood transfusion may be required. To prevent future episodes of hemolysis, the triggers should be avoided. 

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