How we prevent and treat differentiation syndrome in patients with acute promyelocytic leukemia
Open Access
- 1 May 2014
- journal article
- case report
- Published by American Society of Hematology in Blood
- Vol. 123 (18), 2777-2782
- https://doi.org/10.1182/blood-2013-10-512640
Abstract
Differentiation syndrome (DS), formerly known as retinoic acid syndrome, is a relatively common and potentially severe complication seen in patients with acute promyelocytic leukemia treated with all-trans retinoic acid and/or arsenic trioxide. The full-blown syndrome consists of unexplained fever, weight gain, dyspnea with pulmonary infiltrates, pleuropericardial effusion, hypotension, and renal failure. Most measures currently used for management of DS have very little evidence-based support, and therefore, many remain controversial. Despite the lack of evidence supporting DS prophylaxis, several groups have adopted a preventive strategy with corticosteroids, especially for patients with leukocyte levels higher than from 5 to 10 × 109/L. DS diagnosis should be suspected in the presence of any of the above-mentioned signs and symptoms, and preemptive treatment with dexamethasone should be started immediately. Other supportive measures can also be crucial for the correct management of DS, especially in those patients with life-threatening complications. Temporary discontinuation of all-trans retinoic acid or arsenic trioxide is indicated only for patients in very poor clinical condition or with severe renal or pulmonary dysfunction, sometimes requiring admission to the intensive care unit. Recognition of specific biomarkers and a better understanding of DS pathogenesis can be helpful for the development of specific therapies to counteract DS in a timely manner.Keywords
This publication has 28 references indexed in Scilit:
- Retinoic Acid and Arsenic Trioxide for Acute Promyelocytic LeukemiaThe New England Journal of Medicine, 2013
- All-trans-retinoic acid, idarubicin, and IV arsenic trioxide as initial therapy in acute promyelocytic leukemia (APML4)Blood, 2012
- Front-line treatment of acute promyelocytic leukemia with AIDA induction followed by risk-adapted consolidation for adults younger than 61 years: results of the AIDA-2000 trial of the GIMEMA GroupBlood, 2010
- Risk-adapted treatment of acute promyelocytic leukemia based on all-trans retinoic acid and anthracycline with addition of cytarabine in consolidation therapy for high-risk patients: further improvements in treatment outcomeBlood, 2010
- Effective Treatment of Acute Promyelocytic Leukemia With All-Trans-Retinoic Acid, Arsenic Trioxide, and Gemtuzumab OzogamicinJournal of Clinical Oncology, 2009
- Differentiation syndrome in patients with acute promyelocytic leukemia treated with all-trans retinoic acid and anthracycline chemotherapy: characteristics, outcome, and prognostic factorsBlood, 2009
- Is Cytarabine Useful in the Treatment of Acute Promyelocytic Leukemia? Results of a Randomized Trial From the European Acute Promyelocytic Leukemia GroupJournal of Clinical Oncology, 2006
- Risk-adapted treatment of acute promyelocytic leukemia with all-trans-retinoic acid and anthracycline monochemotherapy: a multicenter study by the PETHEMA groupBlood, 2003
- All‐trans‐RETINOIC ACID INCREASES ADHESION TO ENDOTHELIUM OF THE HUMAN PROMYELOCYTIC LEUKAEMIA CELL LINE NB4British Journal of Haematology, 1996
- The "Retinoic Acid Syndrome" in Acute Promyelocytic LeukemiaAnnals of Internal Medicine, 1992