American Board of Internal Medicine (ABIM) Certification Practice Exam

What is the recommended treatment for differentiation syndrome?

1. Immediate surgery

2. Prompt initiation of glucocorticoids and brief interruption of medications

3. Extended hospitalization and observation

4. Increased doses of ATRA and ATO

The correct answer is: Prompt initiation of glucocorticoids and brief interruption of medications

Differentiation syndrome is a potentially serious condition that can occur during treatment for acute promyelocytic leukemia (APL), often associated with the use of all-trans retinoic acid (ATRA) or arsenic trioxide (ATO). The onset of this syndrome is typically marked by symptoms such as fever, weight gain, edema, respiratory distress, and multi-organ dysfunction, which can be life-threatening if not addressed promptly. The recommended management involves the prompt initiation of glucocorticoids, which are critical in reducing inflammation and modulating the immune response that characterizes the syndrome. Glucocorticoids help control the symptoms associated with differentiation syndrome effectively. In addition to glucocorticoid therapy, a brief interruption of ATRA or ATO treatment can also be warranted to manage the patient's symptoms safely without compromising their overall treatment for APL. This approach is preferred over other potential interventions because immediate surgery would not address the underlying causes of the syndrome. Extended hospitalization and observation could be necessary to monitor the patient, but without active treatment with glucocorticoids, this could lead to a deterioration of the patient's condition. Increased doses of ATRA and ATO would potentially worsen differentiation syndrome, as these medications are the very triggers of the