Pathophysiology, clinical features and radiological findings of differentiation syndrome/all-trans-retinoic acid syndrome

• 作者: Carmen Fava Federica Moretti Federico Torre Francesco Asteggiano Giovanna Rege-Cambrin Luciano Cardinale Stefano Ulisciani
• 来源期刊: 世界放射学杂志：英文版（电子版）
• 学科: 医学
• 关键词: Differentiation SYNDROME All-trans-retinoic ACID SYNDROME Chest X-ray and computed tomography Lungleukemic infiltrates Acute PROMYELOCYTIC LEUKAEMIA PROMYELOCYTIC leukemia/retinoic ACID receptor-α
• 年，卷（期）: 2014,（8）
• 页码范围: 583-588
• 页数: 6页
• 分类号: R733.71

摘要

In acute promyelocytic leukemia, differentiation thera-py 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.