All-trans-retinoic acid syndrome
Updates to Article Attributes
All-trans-retinoic acid (ATRA) syndrome, is the more common cause of differentiation syndrome 8. Acute promyelocytic leukaemia (APL) responds to therapeutic levels of this normal plasma constituent by cellular maturation into normal granulocytes.
Organs can become infiltrated by these white cells and damaged by cytokine release causing heart failure, capillary leakage and acute respiratory distress syndrome.
Clinical presentation
It is characterised by fever, respiratory distress, pleural and pericardial effusion, weight gain, and pulmonary opacities on chest radiography.
Radiographic features
Will depend on the system involved although many of the features are described in relation to the lungs.
Plain radiograph
Although the chest radiographic features are not specific, in the context of an appropriate clinical history, they may aid in early recognition of the ATRA syndrome. Recognised chest radiographic features that can be part of the ATRA syndrome include:
In addition, chest radiography may show nodules and consolidation.
CT
Chest CT features are non-specific and similar to congestive heart failure:
cardiomegaly due to myocardial damage and pericardial effusion
systemic venous distension
pleural effusions
lung opacity due to diffuse alveolar damage and haemorrhage
Treatment and Prognosis
Without prompt treatment with corticosteroids, mortality is about 25%. Early intervention with high dose corticosteroids is effective, and those most at risk are given steroid prophylaxis. Acute myeloid leukaemia treated with differentiation agents has a lower mortality rate.
History
It was first described in 1991 by Frankel et al. 1-3,7.
See also
-<p><strong>All-trans-retinoic acid (ATRA) syndrome</strong>, is the more common cause of <strong>differentiation syndrome </strong><sup>8</sup>. <a href="/articles/acute-promyelocytic-leukemia">Acute promyelocytic leukaemia</a> (APL) responds to therapeutic levels of this normal plasma constituent by cellular maturation into normal granulocytes.</p><p>Organs can become infiltrated by these white cells and damaged by cytokine release causing heart failure, capillary leakage and acute respiratory distress syndrome.</p><h4>Clinical presentation</h4><p>It is characterised by fever, respiratory distress, pleural and pericardial effusion, weight gain, and pulmonary opacities on chest radiography.</p><h4>Radiographic features</h4><p>Will depend on the system involved although many of the features are described in relation to the lungs.</p><h5>Plain radiograph</h5><p>Although the chest radiographic features are not specific, in the context of an appropriate clinical history, they may aid in early recognition of the ATRA syndrome. Recognised chest radiographic features that can be part of the ATRA syndrome include: </p><ul>-<li><p><a href="/articles/increased-cardiothoracic-ratio">increased cardiothoracic ratio</a></p></li>-<li><p><a href="/articles/increased-vascular-pedicle-width">increased vascular pedicle width</a></p></li>-<li><p><a href="/articles/ground-glass-opacification-3">ground-glass opacities</a></p></li>-<li><p><a href="/articles/peribronchial-cuffing-1">peribronchial cuffing</a></p></li>-<li><p><a href="/articles/septal-lines-in-lung-1">septal lines</a></p></li>-<li><p><a href="/articles/pleural-effusion">pleural effusion(s)</a> </p></li>-</ul><p>In addition, chest radiography may show nodules and consolidation. </p><h5>CT</h5><p>Chest CT features are non-specific similar to congestive heart failure:</p><ul>-<li><p>cardiomegaly due to myocardial damage and pericardial effusion</p></li>-<li><p>systemic venous distension</p></li>-<li><p>pleural effusions</p></li>-<li><p>lung opacity due to diffuse alveolar damage and haemorrhage</p></li>-</ul><h4>Treatment and Prognosis </h4><p>Without prompt treatment with corticosteroids, mortality is about 25%. Early intervention with high dose corticosteroids is effective, and those most at risk are given steroid prophylaxis.</p><h4>History</h4><p>It was first described in 1991 by Frankel et al. <sup>1-3,7</sup>.</p><p>See also</p><ul>-<li><p><a href="/articles/leukaemia">leukaemia</a></p></li>-<li><p><a href="/articles/leukaemia-thoracic-manifestations">pulmonary manifestations of leukaemia</a></p></li>- +<p><strong>All-trans-retinoic acid (ATRA) syndrome</strong>, is the more common cause of <strong>differentiation syndrome </strong><sup>8</sup>. <a href="/articles/acute-promyelocytic-leukemia">Acute promyelocytic leukaemia</a> (APL) responds to therapeutic levels of this normal plasma constituent by cellular maturation into normal granulocytes.</p><p>Organs can become infiltrated by these white cells and damaged by cytokine release causing <a href="/articles/congestive-cardiac-failure" title="Heart failure">heart failure</a>, capillary leakage and <a href="/articles/acute-respiratory-distress-syndrome-1" title="Acute respiratory distress syndrome">acute respiratory distress syndrome</a>.</p><h4>Clinical presentation</h4><p>It is characterised by fever, respiratory distress, pleural and pericardial effusion, weight gain, and pulmonary opacities on chest radiography.</p><h4>Radiographic features</h4><p>Will depend on the system involved although many of the features are described in relation to the lungs.</p><h5>Plain radiograph</h5><p>Although the chest radiographic features are not specific, in the context of an appropriate clinical history, they may aid in early recognition of the ATRA syndrome. Recognised chest radiographic features that can be part of the ATRA syndrome include: </p><ul>
- +<li><p><a href="/articles/increased-cardiothoracic-ratio">increased cardiothoracic ratio</a></p></li>
- +<li><p><a href="/articles/increased-vascular-pedicle-width">increased vascular pedicle width</a></p></li>
- +<li><p><a href="/articles/ground-glass-opacification-3">ground-glass opacities</a></p></li>
- +<li><p><a href="/articles/peribronchial-cuffing-1">peribronchial cuffing</a></p></li>
- +<li><p><a href="/articles/septal-lines-in-lung-1">septal lines</a></p></li>
- +<li><p><a href="/articles/pleural-effusion">pleural effusion(s)</a> </p></li>
- +</ul><p>In addition, chest radiography may show nodules and consolidation. </p><h5>CT</h5><p>Chest CT features are non-specific and similar to congestive heart failure:</p><ul>
- +<li><p>cardiomegaly due to myocardial damage and pericardial effusion</p></li>
- +<li><p>systemic venous distension</p></li>
- +<li><p>pleural effusions</p></li>
- +<li><p>lung opacity due to diffuse alveolar damage and haemorrhage</p></li>
- +</ul><h4>Treatment and Prognosis</h4><p>Without prompt treatment with corticosteroids, mortality is about 25%. Early intervention with high dose corticosteroids is effective, and those most at risk are given steroid prophylaxis. Acute myeloid leukaemia treated with differentiation agents has a lower mortality rate.</p><h4>History</h4><p>It was first described in 1991 by Frankel et al. <sup>1-3,7</sup>.</p><p>See also</p><ul>
- +<li><p><a href="/articles/leukaemia">leukaemia</a></p></li>
- +<li><p><a href="/articles/leukaemia-thoracic-manifestations">pulmonary manifestations of leukaemia</a></p></li>
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