Initial Management

1. Most important intervention is to quickly determine whether patient is at risk for severe infection or sepsis while assessing ABCs (airway, breathing, circulation). Careful history and physical exam with several sets of vital signs establishes this initial risk evaluation. Hematology consultation with bone marrow aspirate and biopsy is often required to narrow the differential diagnosis.

2. Once determination is made about urgent need for hospitalization, antibiotics, and granulocyte colony-stimulating factor (G-CSF), a more careful workup can begin. History and physical exam can be very informative. A detailed history can uncover prior infections, drug use, hospitalizations, sick siblings, or relatives who died young from infection. Careful physical exam can detect important clues (eg, skeletal malformations of thumb and forearm in Fanconi anemia). Sequential CBCs are critical. Often rechecking the CBC once or twice a week for several weeks points the way to the correct diagnosis. Start with the common diagnoses in the differential and go from there to the more unusual.

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