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Revision as of 02:03, 24 October 2017

Acute promyelocytic leukemia Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Medical Therapy

APL is unique among the leukemias distinguished by its sensitivity to all-trans retinoic acid (ATRA), a derivative of vitamin A. Treatment with ATRA 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.

ATRA therapy is associated with the unique side effect of retinoic acid syndrome. This is associated with the development of dyspnea, fever, 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 allogeneic stem cell transplant. Monitoring for relapse using PCR tests for RARα allows early re-treatment which is successful in many instances.

References

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