Discussion
Diagnosis With Brief Discussion
- Diagnosis
- ATRA (All trans-retinoic acid) syndrome
- Radiologic Findings
- Figs 1-2. Initial chest PA showed clear lung fields. Two weeks after ATRA treatment, chest PA showed enlarged heart and pulmonary congestion. Patchy opacities and septal lines were noted in both lungs. Small amount of bilateral pleural effusion was also noted.
Fig 3. Lung window setting of the chest CT scan showed multifocal peribronchovascular consolidation and ground glass opacities in both lungs.
- Brief Review
Since Frankel et al. first described all trans-retinoic acid (ATRA) syndrome, it has been regarded as a life-threatening complication following ATRA therapy. ATRA syndrome specifically occurs as a result of ATRA usage which can differentiate acute promyelocytic leukemia blasts (M3 in the French-American-British classification of acute myelocytic leukemia) into mature granulocytes.
ATRA syndrome is characterized by fever, respiratory distress, pleural and pericardial effusion, weight gain, and pulmonary infiltrates noted on chest radiography which are also generally associated with an increasing WBC.
The pathophysiology of ATRA syndrome is not completely understood, but it is associated with the increased secretion of various cytokines, particularly interleukin-2, in a manner resembling capillary leak syndrome. The proposed mechanisms may involve changes in the cytokine secretion and adhesive qualities of AML cells during ATRA-induced differentiation.
The major radiographic abnormalities of ATRA syndrome are similar to typical features of cardiogenic pulmonary edema: these include cardiomegaly, vascular pedicle widening, ground-glass opacity, peribronchial cuffing, septal lines and pleural effusion. Nodules and consolidation can also occur.
Due to the nonspecific nature of the aforementioned radiologic features, ATRA syndrome is usually impossible to differentiate from other diseases such as edema, pneumonia or leukemic infiltration without definite clinical history. However, in combination with relevant clinical history, early recognition and quick treatment of the ATRA syndrome can be achieved by withdrawing ATRA combination in conjunction with steroid use.
- Please refer to
Case 316, Case 348, -
KSTR Imaging Conference 2011 Summer Case 5,
- References
- 1. Jung JI, Choi JE, Hahn ST, Kim CC, Park SH. adiologic features of all-trans-retinoic acid syndrome. AJR 2002;178:475-480
2. Frankel SR, Eardley A, Lauwers G, Weiss M, Warrel RP. The "retinoic acid syndrome" in acute promyelocytic leukemia. Ann Intern Med 1992;117:292-296
3. Cardinale L, Asteggiano F, Moretti F, et al. Pathophysiology, clinical features and radiological findings of differentiation syndrome/all-trans-retinoic acid syndrome. World J Radiol. 2014;6:583-588.
- Keywords
- lung, acute myelocytic leukemia, all trans retinoic acid syndrome,