RESUMO
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.
RESUMO
Se describe el caso de una enferma con leucemia aguda promielocítica (LAP) que desarrolló síndrome del ácido transretinoico (SATRA) y se revisa la literatura. El SA TRA se presenta en enfermos con LAP tratados con ácido transretinoico (ATRA). Tiene incidencia de 5% a 27% con mortalidad de hasta 29%. Es secundario al efecto del ATRA sobre la diferenciación de los promielocitos, lo que desencadena respuesta inflamatoria sistémica, daño endotelial con síndrome de fuga capilar y obstrucción de la microcirculación e infiltración tisular. Clínicamente se manifiesta con fiebre, hipotensión, insuficiencia respiratoria, renal y hepática, infiltrados pulmonares, derrame pleural y pericárdico, y edema generalizado. El tratamiento es a base de suspensión del ATRA, medidas de apoyo y esferoides.
We described a patient with acute promyelocytic leukemia (APL) who developed all-trans retinoic acid syndrome (ATRAS) and reviewed the literature. ATRAS presents in patients with APL treated with all-trans retinoic acid (ATRA). It has an incidence from 5%-27% with mortality of 29%. It is secondary to ATRA effect on promyelocyte differentiation, which causes systemic inflammatory response syndrome, endothelium damage with increase in capillary permeability, microcirculation obstruction, and tissue infiltration. ATRAS clinical manifestations are fever, hypotension, respiratory, renal and hepatic insufficiency, lung infiltrates, pleural and pericardic efussion, and generalized edema. Treatment is based on ATRA suspension, support measures, and steroids.
