Review
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World J Radiol. Aug 28, 2014; 6(8): 583-588
Published online Aug 28, 2014. doi: 10.4329/wjr.v6.i8.583
Pathophysiology, clinical features and radiological findings of differentiation syndrome/all-trans-retinoic acid syndrome
Luciano Cardinale, Francesco Asteggiano, Federica Moretti, Federico Torre, Stefano Ulisciani, Carmen Fava, Giovanna Rege-Cambrin
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
Stefano Ulisciani, Carmen Fava, Giovanna Rege-Cambrin, Division of Hematology and Internal Medicine, Department of Clinical and Biological Sciences of University of Turin, AOU S.Luigi Gonzaga, 10043 Orbassano, Torino, Italy
Author contributions: Cardinale L planned the research; Asteggiano F and Torre F collected the materials; Ulisciani S, Fava C, Rege-Cambrin G were involved in the collection of clinical data; Moretti F and Ulisciani S wrote the paper assisted by Fava C; Cardinale L and Rege-Cambrin G reviewed this commentary and supervised its publication; Asteggiano F, Moretti F and Torre F were involved in the language translation.
Correspondence to: Luciano Cardinale, PhD, Institute of Radiology, Department of Clinical and Biological Sciences, University of Turin, AOU S.Luigi Gonzaga, Regione Gonzole 10, 10043 Orbassano, Torino, Italy. luciano.cardinale@gmail.com
Telephone: +39-11-90261 Fax: +39-11-9026303
Received: December 28, 2013
Revised: July 9, 2014
Accepted: July 17, 2014
Published online: August 28, 2014
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.

Keywords: Differentiation syndrome, All-trans-retinoic acid syndrome, Chest X-ray and computed tomography, Lungleukemic infiltrates, Acute promyelocytic leukaemia, Promyelocytic leukemia/retinoic acid receptor-α

Core tip: Aim of this review is to illustrate the spectrum of chest imaging findings which lead to suspect a diagnosis of differentiation syndrome, which arise in patients suffering of Acute Promyelocytic Leukemia after treatment with all-trans-retinoic acid or other differentiating drugs, in order to facilitate the differential diagnosis with other life-threatening pulmonary complications occurring in this subset of highly immunocompromised patients.