A 5-year-old child with sickle cell disease presents with sudden onset of pallor, weakness, and rapid pulse. His hemoglobin drops from a baseline of 9 g/dL to 3.5 g/dL within hours. The spleen is massively enlarged and tender. Reticulocyte count is 15%. What is the immediate life-saving treatment?
- A Prompt blood transfusion (simple transfusion) to correct anemia ✓
- B Hydroxyurea urgently to reduce sickling
- C Emergency splenectomy
- D Exchange transfusion to reduce HbS percentage to <30%
Explanation
The clinical picture describes acute splenic sequestration crisis (ASSC) — the most common cause of rapid-onset severe anemia and death in young children with sickle cell disease. Sickled red cells obstruct the splenic sinusoids, causing massive trapping of blood in the spleen and acute anemia. The high reticulocyte count confirms active erythropoiesis (splenic failure is not the cause). Immediate simple blood transfusion to restore hemoglobin and hemodynamic stability is life-saving. Hydroxyurea is a chronic preventive therapy. Exchange transfusion is used for cerebrovascular accidents and acute chest syndrome with severe hypoxia, not ASSC.
Reference: Ghai Essential Pediatrics, 10th ed.
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Written and medically reviewed by the StethoPrep medical team.