| Differentiation syndrome in promyelocytic leukemia: clinical presentation, pathogenesis and treatment. | |
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MedLine Citation:
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PMID: 22110898 Owner: NLM Status: In-Data-Review |
Abstract/OtherAbstract:
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Differentiation syndrome (DS) represents a life-threatening complication in patients with acute promyelocytic leukemia (APL) undergoing induction therapy with all-trans retinoic acid (ATRA) or arsenic trioxide (ATO). It affected about 20-25% of all patients and so far there are no 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-alveolar pulmonary infiltration by maturing myeloid cells, endothelial cell damage, intra-alveolar edema, inter-alveolar hemorrhage, and fibrinous exsudates. DS pathogenesis is not completely understood, but it is 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, its recognition and the prompt initiation of 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. |
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Authors:
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E M Rego; G C De Santis |
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Publication Detail:
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Type: Journal Article Date: 2011-10-24 |
Journal Detail:
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Title: Mediterranean journal of hematology and infectious diseases Volume: 3 ISSN: 2035-3006 ISO Abbreviation: Mediterr J Hematol Infect Dis Publication Date: 2011 |
Date Detail:
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Created Date: 2011-11-23 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 101530512 Medline TA: Mediterr J Hematol Infect Dis Country: Italy |
Other Details:
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Languages: eng Pagination: e2011048 Citation Subset: - |
Affiliation:
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National Institute of Science and Technology in Stem Cell and Cell Therapy, Division of Oncology/Hematology, Department of Internal Medicine, Medical School of Ribeirão Preto, University of São Paulo. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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