Retinoic acid syndrome: Difference between revisions
m (Robot: Changing Category:Diseases to Category:Disease) |
No edit summary |
||
| Line 1: | Line 1: | ||
__NOTOC__ | |||
{{SI}} | |||
{{CMG}} | |||
{{SK}} APML differentiation syndrome; APL differentiation syndrome | |||
==Overview== | |||
'''Retinoic acid syndrome''' (RAS) is a potentially life-threatening complication observed in patients with [[acute promyelocytic leukemia]] (APML) and first thought to be specifically associated with [[Tretinoin|all-trans retinoic acid]] (ATRA) (also known as tretinoin) treatment.<ref name="pmid18945746">{{cite journal |author=Breccia M, Latagliata R, Carmosino I, ''et al.'' |title=Clinical and biological features of acute promyelocytic leukemia patients developing retinoic acid syndrome during induction treatment with all-trans retinoic acid and idarubicin |journal=Haematologica |volume=93 |issue=12 |pages=1918–20 |date=December 2008 |pmid=18945746 |doi=10.3324/haematol.13510 |url=http://www.haematologica.org/cgi/pmidlookup?view=long&pmid=18945746}}</ref> Subsequently it was recognized that so-called RAS appeared in APML patients who had been treated with another highly efficacious drug, [[arsenic trioxide]], and yet did not appear in patients treated with [[tretinoin]] for other disorders. These facts and others support the notion that RAS depends on the presence of the malignant promyelocytes. This has led to the growing deprecation of the term 'retinoic acid syndrome' and to an increasing use of the term '''differentiation syndrome''' to signify this APML treatment complication.<ref>{{cite web|last=Weinberger|first=Steven|title=Differentiation (retinoic acid) syndrome|url=http://www.uptodate.com/contents/differentiation-retinoic-acid-syndrome|accessdate=10 March 2011}}</ref> | |||
==Presentation== | |||
The syndrome is characterized by dyspnea, fever, weight gain, hypotension, and pulmonary infiltrates. This is effectively treated by giving [[dexamethasone]] and holding ATRA (or arsenic trioxide) in severe cases. An elevated white count is sometimes associated with this syndrome, but is not a prerequisite. | |||
Once RAS has resolved, pro-differentiation chemotherapy can be resumed. | |||
==Etiology== | |||
The etiology of RAS is not clear cut. Several causes have been speculated including a [[capillary leak syndrome]] from [[cytokine]] release from the differentiating myeloid cells. Alternatively, ATRA may cause the maturing myeloid cells to acquire the ability to infiltrate organs such as the lung. | The etiology of RAS is not clear cut. Several causes have been speculated including a [[capillary leak syndrome]] from [[cytokine]] release from the differentiating myeloid cells. Alternatively, ATRA may cause the maturing myeloid cells to acquire the ability to infiltrate organs such as the lung. | ||
Mediation by [[cathepsin G]] has been suggested.<ref name="pmid11840279">{{cite journal |author=Tallman MS |title=Retinoic acid syndrome: a problem of the past? |journal=Leukemia |volume=16 |issue=2 |pages=160–1 |date=February 2002 |pmid=11840279 |doi=10.1038/sj.leu.2402344}}</ref> | |||
==Treatment== | |||
The treatment of RAS usually involves administering dexamethasone IV, with the dosage usually 10 mg BID for ten days. It is important for patients to discontinue the use of tretinoin due to the elevation of WBC and possible low blood oxygen. | |||
==See also== | |||
* [[Hypervitaminosis A]] | |||
{{ | ==References== | ||
{{reflist|2}} | |||
[[Category: | [[Category:Paraneoplastic syndromes]] | ||
Revision as of 16:14, 20 January 2015
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords: APML differentiation syndrome; APL differentiation syndrome
Overview
Retinoic acid syndrome (RAS) is a potentially life-threatening complication observed in patients with acute promyelocytic leukemia (APML) and first thought to be specifically associated with all-trans retinoic acid (ATRA) (also known as tretinoin) treatment.[1] Subsequently it was recognized that so-called RAS appeared in APML patients who had been treated with another highly efficacious drug, arsenic trioxide, and yet did not appear in patients treated with tretinoin for other disorders. These facts and others support the notion that RAS depends on the presence of the malignant promyelocytes. This has led to the growing deprecation of the term 'retinoic acid syndrome' and to an increasing use of the term differentiation syndrome to signify this APML treatment complication.[2]
Presentation
The syndrome is characterized by dyspnea, fever, weight gain, hypotension, and pulmonary infiltrates. This is effectively treated by giving dexamethasone and holding ATRA (or arsenic trioxide) in severe cases. An elevated white count is sometimes associated with this syndrome, but is not a prerequisite.
Once RAS has resolved, pro-differentiation chemotherapy can be resumed.
Etiology
The etiology of RAS is not clear cut. Several causes have been speculated including a capillary leak syndrome from cytokine release from the differentiating myeloid cells. Alternatively, ATRA may cause the maturing myeloid cells to acquire the ability to infiltrate organs such as the lung.
Mediation by cathepsin G has been suggested.[3]
Treatment
The treatment of RAS usually involves administering dexamethasone IV, with the dosage usually 10 mg BID for ten days. It is important for patients to discontinue the use of tretinoin due to the elevation of WBC and possible low blood oxygen.
See also
References
- ↑ Breccia M, Latagliata R, Carmosino I; et al. (December 2008). "Clinical and biological features of acute promyelocytic leukemia patients developing retinoic acid syndrome during induction treatment with all-trans retinoic acid and idarubicin". Haematologica. 93 (12): 1918–20. doi:10.3324/haematol.13510. PMID 18945746.
- ↑ Weinberger, Steven. "Differentiation (retinoic acid) syndrome". Retrieved 10 March 2011.
- ↑ Tallman MS (February 2002). "Retinoic acid syndrome: a problem of the past?". Leukemia. 16 (2): 160–1. doi:10.1038/sj.leu.2402344. PMID 11840279.