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Difficulty: MediumHematology and Oncology

A 22-year-old man with sickle cell disease comes to the urgent care center due to a 3-day history of productive cough, fever, and severe, sharp chest pain. He also reports increasing difficulty breathing over the last 24 hours. The patient takes folic acid and hydroxyurea daily. His temperature is 38.8°C (101.8°F), blood pressure is 114/72 mm Hg, pulse is 122/min, and respirations are 28/min. Pulse oximetry shows an oxygen saturation of 89% on room air. Physical examination reveals scleral icterus. Lung auscultation demonstrates decreased breath sounds and crackles in the right lower lung field.

Laboratory test results are as follows:

TestResult
Hemoglobin7.1 g/dL
Leukocyte count19,200/mm³
Platelet count480,000/mm³
Reticulocyte count14%

Arterial blood gas analysis on room air reveals:

TestResult
pH7.49
PaCO228 mm Hg
PaO260 mm Hg

A chest radiograph displays a new opacity in the right lower lobe. Which of the following is the most appropriate next step in the management of this patient?

  1. Ceftriaxone and azithromycinAnswer
  2. B
    Furosemide
  3. C
    Meropenem and vancomycin
  4. D
    Sodium bicarbonate
  5. E
    Thyroid-stimulating hormone level

Answer

The correct management is to initiate empiric antibiotic therapy with ceftriaxone and azithromycin.
The patient's presentation of fever, chest pain, new pulmonary opacity, and hypoxemia in the setting of sickle cell disease is diagnostic of acute chest syndrome (ACS). ACS is a leading cause of mortality in sickle cell patients and requires immediate, aggressive treatment. Initial management includes supplemental oxygen, pain control, cautious intravenous fluids, and broad empiric antibiotics covering typical and atypical respiratory pathogens, as infection is a frequent trigger for the syndrome. A third-generation cephalosporin (e.g., ceftriaxone) plus a macrolide (e.g., azithromycin) is the standard antibiotic regimen.

Step-by-Step Solution

1
Evaluate the patient's clinical presentation and vital signs
Fever, tachycardia, tachypnea, hypoxemia, and chest pain in a patient with sickle cell disease
To identify the clinical syndrome and assess severity
2
Analyze the laboratory and radiographic findings
Anemia, leukocytosis, and a new lower lobe pulmonary infiltrate
To differentiate acute chest syndrome from an uncomplicated vaso-occlusive crisis
3
Determine the appropriate management for acute chest syndrome
Empiric coverage for community-acquired typical and atypical respiratory pathogens is required
Infection is a common trigger for acute chest syndrome, and clinical outcomes improve with prompt antibiotic administration

Key Concept

Diagnosis and management of acute chest syndrome in sickle cell disease
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Topics

Hematology and Oncology

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