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Retinoic acid and APL
Acute Promyelocytic Leukemia (APL)
is unique among the leukemias distinguished by its sensitivity to
all-trans retinoic acid
(ATRA), a derivative of vitamin A.
is a form of "differentiation therapy."
It activates the retinoid receptor
of the immature leukemic promyelocytes into mature granulocytes.
is typically combined with
based chemotherapy resulting in a clinical remission in approximately 90% of patients. However,
cannot eliminate the leukemic clone.
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
allogenic stem cell transplant
Better with the combination of
and chemotherapy than chemotherapy alone in newly diagnosed
+ chemotherapy makes for a slightly higher rate of complete remissions while allowing significantly fewer relapses. Maintenance treatment with
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.
more than 90% of patients with newly diagnosed
today can achieve complete remission
about 75% can be cured by the combination of