APL is unique among the leukemias distinguished by its sensitivity to
all-trans retinoic acid (ATRA), a derivative of
vitamin A. Treatment with ATRA causes differentiation of the immature leukemic promyelocytes into mature granulocytes. ATRA is typically combined with
anthracycline based chemotherapy resulting in a clinical remission in approximately 90% of patients.
ATRA therapy is associated with the unique side effect of
retinoic acid syndrome. This is associated with the development of dyspnea, fever, weight gain, peripheral edema and is treated with
dexamethasone. The etiology of retinoic acid syndrome has been attributed to capillary leak syndrome from cytokine release from the differentiating promyelocytes.
Ans: A