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Difficulty: EasyPediatric Hematology and Oncology

A 2-year-old boy with sickle cell disease (HbSS) is brought to the emergency department by his parents due to the sudden onset of severe lethargy, pallor, and abdominal swelling. His parents report that he was playing normally this morning but became progressively weak and poorly responsive over the past 3 hours. His temperature is 37.1°C (98.8°F), heart rate is 165/min, respiratory rate is 40/min, and blood pressure is 75/40 mm Hg. Physical examination reveals marked conjunctival pallor and a massively enlarged, firm spleen extending into the left lower quadrant. Laboratory studies show a hemoglobin of 3.8 g/dL, platelets of 80,000/mm³, and a reticulocyte count of 18%. What is the most appropriate next step in the management of this patient?

  1. A
    Immediate transport to the radiology department for a computed tomography scan of the abdomen
  2. B
    Intravenous administration of broad-spectrum antibiotics for suspected occult bacteremia
  3. Rapid administration of isotonic intravenous fluids and a packed red blood cell transfusionAnswer
  4. D
    Immediate surgical consultation for an exploratory laparotomy to rule out bowel obstruction
  5. E
    Intravenous administration of corticosteroids and bronchodilators for an acute cardiopulmonary event

Answer

Rapid administration of isotonic intravenous fluids and a packed red blood cell transfusion
The correct answer is rapid administration of isotonic intravenous fluids and a packed red blood cell transfusion. This patient's presentation of sudden-onset severe anemia, hypovolemic shock (tachycardia, hypotension), an elevated reticulocyte count, and a rapidly enlarging spleen is classic for acute splenic sequestration crisis, a life-threatening complication of sickle cell disease. Immediate volume resuscitation and blood transfusion are required to restore intravascular volume and oxygen-carrying capacity.

Step-by-Step Solution

1
Identify the patient's underlying condition and presenting symptoms.
The patient has sickle cell disease and presents with hypovolemic shock (tachycardia, hypotension), severe anemia, elevated reticulocyte count, and a rapidly enlarging spleen.
Recognizing this classic triad establishes the diagnosis of acute splenic sequestration crisis.
2
Determine the most appropriate immediate management.
Immediate volume resuscitation with isotonic fluids and a packed red blood cell transfusion is required.
The condition involves massive pooling of blood in the spleen, leading to life-threatening intravascular volume depletion that must be rapidly corrected to prevent cardiovascular collapse.

Key Concept

Acute splenic sequestration crisis is a life-threatening complication in young children with sickle cell disease characterized by severe anemia, rapidly enlarging splenomegaly, and hypovolemic shock, requiring immediate fluid resuscitation and blood transfusion.
Estimated Time:1m 0s
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Topics

Pediatric Hematology and Oncology

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