Retinoic acid syndrome: Difference between revisions
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==Overview== | ==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> | '''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> | ||
==Pathophysiology== | |||
Several mechanisms have been speculated including a [[capillary leak syndrome]] from [[cytokine]] release from the differentiating myeloid cells. Mediation by [[cathepsin G]] has also 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> | |||
==Causes== | ==Causes== | ||
The etiology of RAS remains unclear | The etiology of RAS remains unclear. Alternatively, [[ATRA|all-trans retinoic acid (ATRA)]] and [[arsenic trioxide]] may cause the maturing myeloid cells to acquire the ability to infiltrate organs such as the lung. | ||
==Diagnosis== | ==Diagnosis== | ||
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===Laboratory Findings=== | ===Laboratory Findings=== | ||
An elevated white count is sometimes associated with this syndrome, but is not a prerequisite. | An elevated white count is sometimes associated with this syndrome, but is not a prerequisite. | ||
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Once RAS has resolved, pro-differentiation chemotherapy can be resumed. | Once RAS has resolved, pro-differentiation chemotherapy can be resumed. | ||
==References== | ==References== | ||
Latest revision as of 21:22, 30 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]
Pathophysiology
Several mechanisms have been speculated including a capillary leak syndrome from cytokine release from the differentiating myeloid cells. Mediation by cathepsin G has also been suggested.[3]
Causes
The etiology of RAS remains unclear. Alternatively, all-trans retinoic acid (ATRA) and arsenic trioxide may cause the maturing myeloid cells to acquire the ability to infiltrate organs such as the lung.
Diagnosis
Symptoms
The syndrome is characterized by dyspnea, fever, weight gain, hypotension, and pulmonary infiltrates.
Laboratory Findings
An elevated white count is sometimes associated with this syndrome, but is not a prerequisite.
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.
Once RAS has resolved, pro-differentiation chemotherapy can be resumed.
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.