Differentiation syndrome in acute promyelocytic leukaemia Review


Authors: Stahl, M.; Tallman, M. S.
Review Title: Differentiation syndrome in acute promyelocytic leukaemia
Abstract: Acute promyelocytic leukaemia differentiation syndrome (APL DS) is seen when patients with APL are treated with all-trans retinoic acid (ATRA) and/or arsenic trioxide (ATO). Presenting symptoms are varied but frequently include dyspnoea, unexplained fever, weight gain >5 kg, unexplained hypotension, acute renal failure and a chest radiograph demonstrating pulmonary infiltrates or pleural or pericardial effusion. Immediate treatment with steroids at the first clinical suspicion is recommended and ATRA/ATO should be stopped in severe cases or if there is no response to treatment. The utility of steroid prophylaxis in order to prevent APL DS is less certain. Here we provide a detailed review of the pathogenesis, clinical signs and symptoms as well as management and prophylaxis strategies of APL DS. © 2019 British Society for Haematology and John Wiley & Sons Ltd
Keywords: prednisone; clinical feature; pathogenesis; review; outcome assessment; interleukin 1beta; interleukin 8; dexamethasone; steroid; arsenic trioxide; prothrombin time; disease severity; promyelocytic leukemia; interleukin 6; cytokine production; methylprednisolone; thorax radiography; symptomatology; leukocyte count; treatment withdrawal; fibrinogen; partial thromboplastin time; tumor necrosis factor; systemic inflammatory response syndrome; lymphocyte function associated antigen 1; all-trans retinoic acid; retinoic acid syndrome; human; priority journal; acute promyelocytic leukaemia; differentiation syndrome; prognostic assessment; retinoid acid syndrome; very late activation antigen
Journal Title: British Journal of Haematology
Volume: 187
Issue: 2
ISSN: 0007-1048
Publisher: John Wiley & Sons  
Date Published: 2019-10-01
Start Page: 157
End Page: 162
Language: English
DOI: 10.1111/bjh.16151
PUBMED: 31410848
PROVIDER: scopus
DOI/URL:
Notes: Review -- Export Date: 1 November 2019 -- Source: Scopus
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  1. Martin Stuart Tallman
    649 Tallman
  2. Maximilian Stahl
    42 Stahl