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Retinoic acid and APL
Acute Promyelocytic Leukemia (APL)
Author:
elena comino
Date:
04/10/2007
Description
TREATMENT
APL
is unique among the leukemias distinguished by its sensitivity to
all-trans retinoic acid
(ATRA), a derivative of vitamin A.
ATRA
is a form of "differentiation therapy."
It activates the retinoid receptor
RAR
and causes
differentiation
of the immature leukemic promyelocytes into mature granulocytes.
ATRA
is typically combined with
anthracycline
based chemotherapy resulting in a clinical remission in approximately 90% of patients. However,
ATRA
cannot eliminate the leukemic clone.
ATRA
therapy is associated with the unique side effect of
retinoic acid syndrome
. This is associated with the development of dyspnea, fever, hypotension, weight gain, peripheral edema and is treated with dexamethasone.
The etiology of retinoic acid syndrome has been attributed to capillary leak syndrome from cytokine release from the differentiating promyelocytes.
Treatment options for patients with
relapsed disease
include
arsenic trioxide
and
allogenic stem cell transplant
.
PROGNOSIS
Better with the combination of
ATRA
and chemotherapy than chemotherapy alone in newly diagnosed
APL,
because
ATRA
+ chemotherapy makes for a slightly higher rate of complete remissions while allowing significantly fewer relapses. Maintenance treatment with
ATRA,
and possibly with low-dose chemotherapy, further reduces the incidence of relapse.
Depends on a number of factors including the white blood cell (WBC) count at the time of diagnosis, etc.
Overall,
more than 90% of patients with newly diagnosed
APL
today can achieve complete remission
about 75% can be cured by the combination of
ATRA
and chemotherapy.