Acute promyelocytic leukemia: from highly fatal to highly curable.

Background All-trans-retinoic acid induces complete remission in acute promyelocytic leukemia. However, it is not clear whether induction therapy with all-trans-retinoic acid is superior to chemotherapy alone or whether maintenance treatment with all-trans-retinoic acid improves outcome. Methods Three hundred forty-six patients with previously untreated acute promyelocytic leukemia were randomly assigned to receive all-trans-retinoic acid or daunorubicin plus cytarabine as induction treatment. Patients who had a complete remission received consolidation therapy consisting of one cycle of treatment identical to the induction chemotherapy, then high-dose cytarabine plus daunorubicin. Patients still in complete remission after two cycles of consolidation therapy were then randomly assigned to maintenance treatment with all-trans-retinoic acid or to observation. Results Of the 174 patients treated with chemotherapy, 120 (69 percent) had a complete remission, as did 124 of the 172 (72 percent) given all-trans-...

https://www.nejm.org/doi/pdf/10.1056/NEJM199710093371501

All-trans-Retinoic Acid in Acute Promyelocytic Leukemia

Deconstructing a Disease: RAR, Its Fusion Partners, and Their Roles in the Pathogenesis of Acute Promyelocytic Leukemia

Acute promyelocytic leukemia (APL) is a distinct subtype of acute myeloid leukemia (AML), identified by the French-American-British (FAB) classification as AML-M3. It accounts for approx 10–15% of all AML cases in most reports (1) but can be as high as 32% in some areas of China (2) and 46% among AML patients of American-Mexican descent (3). Clinically, APL is associated with a high incidence of coagulopathy, either disseminated intravascular coagulation (DIC) or hyperfibrinolysis, which are often aggravated during chemotherapy and result in death at an early stage of treatment. Cytogenetically, APL is characterized in 95% of the cases by a balanced reciprocal translocation between chromosomes 15 and 17, t(15;17)(q22;q21), which leads to the formation of two fusion genes, promyelocytic leukemia—retinoic acid receptor α(PML-RARα) or RARα-PML, the former being considered to play a crucial role in leukemogenesis (4).

Acute Promyelocytic Leukemia

A Randomized Comparison of All Transretinoic Acid (ATRA) Followed by Chemotherapy and ATRA Plus Chemotherapy and the Role of Maintenance Therapy in Newly Diagnosed Acute Promyelocytic Leukemia

A clinical and experimental study on all-trans retinoic acid-treated acute promyelocytic leukemia patients

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A clinical and experimental study on all-trans retinoic acid-treated acute promyelocytic leukemia patients