How is differentiation syndrome treated in acute promyelocytic leukemia (APL)?

Updated: May 03, 2019
  • Author: Sandy D Kotiah, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

A high degree of clinical suspicion for differentiation syndrome should always lead to initiation of dexamethasone therapy at the first sign of symptoms of respiratory compromise. Temporary discontinuation of ATRA therapy should be considered until hypoxia resolves. Cytoreduction, hydroxyurea, and anthracycline are recommended for difficult-to-treat differentiation syndrome. [14]

Hyperleukocytosis is common in differentiation syndrome, but leukocyte counts may also be normal. Gemtuzumab or anthracyclines (idarubicin and daunorubicin) can be used to control the leukocyte count if necessary. [10]

In those patients who are not treated initially with chemotherapy, a rapid increase in WBC count may follow ATRA use. These patients should be promptly treated with chemotherapy to avoid clinical hyperleukocytosis.


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