title: Pathophysiology, clinical features and radiological findings of differentiation syndrome/all-trans-retinoic acid syndrome.
abstract: In acute promyelocytic leukemia, differentiation therapy based on all-trans-retinoic acid can be complicated by the development of a differentiation syndrome (DS). DS is a life-threatening complication, characterized by respiratory distress, unexplained fever, weight gain, interstitial lung infiltrates, pleural or pericardial effusions, hypotension and acute renal failure. The diagnosis of DS is made on clinical grounds and has proven to be difficult, because none of the symptoms is pathognomonic for the syndrome without any definitive diagnostic criteria. As DS can have subtle signs and symptoms at presentation but progress rapidly, end-stage DS clinical picture resembles the acute respiratory distress syndrome with extremely poor prognosis; so it is of absolute importance to be conscious of these complications and initiate therapy as soon as it was suspected. The radiologic appearance resembles the typical features of cardiogenic pulmonary edema. Diagnosis of DS remains a great skill for radiologists and haematologist but it is of an utmost importance the cooperation in suspect DS, detect the early signs of DS, examine the patients' behaviour and rapidly detect the complications.
authors.affiliation: Luciano Cardinale, Francesco Asteggiano, Federica Moretti, Federico Torre, Institute of Radiology, Department of Clinical and Biological Sciences, University of Turin, AOU S.Luigi Gonzaga, 10043 Orbassano, Torino, Italy.
authors.forename: Luciano
authors.initials: L
authors.lastname: Cardinale
authors.affiliation: Luciano Cardinale, Francesco Asteggiano, Federica Moretti, Federico Torre, Institute of Radiology, Department of Clinical and Biological Sciences, University of Turin, AOU S.Luigi Gonzaga, 10043 Orbassano, Torino, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Asteggiano
authors.affiliation: Luciano Cardinale, Francesco Asteggiano, Federica Moretti, Federico Torre, Institute of Radiology, Department of Clinical and Biological Sciences, University of Turin, AOU S.Luigi Gonzaga, 10043 Orbassano, Torino, Italy.
authors.forename: Federica
authors.initials: F
authors.lastname: Moretti
authors.affiliation: Luciano Cardinale, Francesco Asteggiano, Federica Moretti, Federico Torre, Institute of Radiology, Department of Clinical and Biological Sciences, University of Turin, AOU S.Luigi Gonzaga, 10043 Orbassano, Torino, Italy.
authors.forename: Federico
authors.initials: F
authors.lastname: Torre
authors.affiliation: Luciano Cardinale, Francesco Asteggiano, Federica Moretti, Federico Torre, Institute of Radiology, Department of Clinical and Biological Sciences, University of Turin, AOU S.Luigi Gonzaga, 10043 Orbassano, Torino, Italy.
authors.forename: Stefano
authors.initials: S
authors.lastname: Ulisciani
authors.affiliation: Luciano Cardinale, Francesco Asteggiano, Federica Moretti, Federico Torre, Institute of Radiology, Department of Clinical and Biological Sciences, University of Turin, AOU S.Luigi Gonzaga, 10043 Orbassano, Torino, Italy.
authors.forename: Carmen
authors.initials: C
authors.lastname: Fava
authors.affiliation: Luciano Cardinale, Francesco Asteggiano, Federica Moretti, Federico Torre, Institute of Radiology, Department of Clinical and Biological Sciences, University of Turin, AOU S.Luigi Gonzaga, 10043 Orbassano, Torino, Italy.
authors.forename: Giovanna
authors.initials: G
authors.lastname: Rege-Cambrin
keywords: Acute promyelocytic leukaemia; All-trans-retinoic acid syndrome; Chest X-ray and computed tomography; Differentiation syndrome; Lungleukemic infiltrates; Promyelocytic leukemia/retinoic acid receptor-α