What is differentiation syndrome? What role does age appear to play in the risk of developing it and in strategies for managing it?
Differentiation syndrome is a serious side effect that may occur in patients with APL who have been treated with certain anticancer drugs. Differentiation syndrome usually occurs within a week or 2 of starting treatment. It is caused by a large, rapid release of cytokines [immune substances] from leukemia cells. The most common symptoms include fever; cough; shortness of breath; weight gain; swelling of the arms, legs, and neck; build-up of excess fluid around the heart and lungs; low blood pressure; and kidney failure. Differentiation syndrome can be life-threatening if not recognized and treated early.
Some evidence suggests older adults may be at a higher risk for developing differentiation syndrome and may be less likely to tolerate it. A risk factor is kidney dysfunction, which is more common in older adults.
It is not clear that management should differ by age, but vigilance is critical. The use of prophylactic steroids is considered for high-risk patients [high white cell count or kidney disease]. The treatment for differentiation syndrome involves rapid use of steroids.
Does the management of infections differ in older people with APL?
There is no clear data to support a different management of infection prevention for older adults, although preventive antibiotics can be considered as older adults are at a higher risk for infectious complications. However, drug interactions need to be carefully considered in this context.
Guiding clinicians toward better treatment of APL
Why did you decide to formulate these recommendations now?
It is particularly important to draw attention to the management of older adults with APL given the availability of effective non–chemotherapy based therapies and the large distinction between expected outcomes with APL vs. other types of acute leukemia in this population. This diagnosis should not be missed. Further, we highlight the importance of ensuring that older adults are included in trials to provide best evidence for both treatment choice and supportive care management.
How do you see these recommendations affecting clinical practice?
We want to emphasize that advanced age should not preclude treatment, which can have meaningful benefit with expectation of remission and quality time gained.
We hope that these recommendations provide a useful blueprint for guiding the management of older adults, particularly consolidating information to help inform treatment for those patients older than 75 years that can provide best estimates of side effects and benefits when making a decision with patients. We also hope that these recommendations will be used to educate providers on the importance of looking for this diagnosis in our older patients.
From a practical standpoint, it will be important that this information gets to those providers who are making the referrals to oncologists, which can include primary care physicians and emergency room providers, to ensure prompt diagnostic workup. Treatment decisions can only be made once a diagnosis has been recognized, and time is critical with this disease.
Dr. Klepin disclosed a consultancy for Genentech and Pfizer and is a contributor to UpToDate.
A version of this article originally appeared on Medscape.com.