Differentiation Syndrome Pathogenesis and Treatment

Differentiation syndrome (DS) represents a life acute promyelocytic leukemia (APL) undergoing induction therapy with all (ATRA) or arsenic trioxide (ATO). It affected about 20 definitive diagnostic criteria. Clinically, DS is characterized by weight gain, fever not attributable to infection, respiratory distress, cardiac involvement, hypotension, and/or acute renal failure. At the histological point of view, there is an extensive interstitial and intra infiltration by maturing myeloid cells, endothelial cell damage, intra hemorrhage, and fibrinous exsudates. DS pathogenesis is not completely understood, b believed that an excessive inflammatory response is the main phenomenon involved, which results in increased production of chemokines and expression of adhesion molecules on APL cells. Due to the high morbidity and mortality associated with DS, it the treatment is of utmost importance. Dexamethasone is considered the mainstay of treatment of DS, and the recommended dose is 10 mg twice daily by intravenous route until resolution of DS. In severe cases (respiratory or acute renal failure) it is recommended the discontinuation of ATRA or ATO until recovery. Introduction. Contemporary treatment of acute promyelocytic leukemia (APL) consists of a combination of all-trans retinoic acid (ATRA) with anthracycline-containing chemotherapy, or arsenic trioxide (ATO). These modalities of treatment lead to complete remission rates greater than 90% and cure rates of approximately 80%, in contrast with results www.mjhid.org ISSN 2035-3006 in Promyelocytic Leukemia: Clinical Presentation, School of Ribeirão Preto, University of São Paulo