DS is a potentially fatal complication of treatment with differentiating agents. It is caused by a rapid proliferation and differentiation of myeloid precursor cells, which in turn triggers a rapid release of inflammatory cytokines that can lead to a variety of symptoms.
- The severity of DS correlates with the number of clinical features present:
- Severe: ≥4 clinical features
- Moderate: 3 clinical features
- Indeterminate: 1 to 2 clinical features
- Less commonly, DS may be associated with pulmonary hemorrhage or acute febrile neutrophilic dermatosis
- Patients are often treated with corticosteroids