Objective: To study the clinical features of childhood acute promyelocytic leukemia (APL) and to analyze the survival and prognostic factors and efficacy and safety of combined treatment with all-trans retinoic acid (ATRA) and anthracycline.
Method: The clinical features of 37 children with newly diagnosed APL hospitalized in our center during January 2005 to February 2009 were retrospectively analyzed.
Result: Thirty percent of patients were at low risk, 43% patients were at intermediate risk, 27% patients were at high risk. Sixty percent of patients had DIC. Retinoic acid syndrome (RAS) was present in 2 patients (6%). Death during induction occurred in 3 patients (8%). Complete remission (CR) was achieved in 83.7% of patients. The patients in high risk group had higher risk than those in intermediate and low risk group (P = 0.029). The time to achieve CR was not significantly different (P = 0.612). Idarubicin had no advantage compared with daunorubicin in time to achieve CR (P = 0.628). Survival rates were calculated using Kaplan-Meier statistical method, and 2 years event-free survival (EFS) rate was 81%, the 2-year EFS rate was 100% for low-risk group, 81% for intermediate-risk group, and 60% for high-risk group.
Conclusion: Using combined chemotherapy with ATRA and anthracyclines had the following advantages: high CR rate, high long-time survival rate and low side effect. DIC remained the main complication among patients receiving induction treatment. Initial WBC count and platelet count are important prognostic factors which might be useful in prognostication and treatment planning.
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Curr Top Dev Biol
January 2025
Department of Pharmacology and Cleveland Center for Membrane and Structural Biology, Case Western Reserve University, Cleveland, OH, United States.
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Université de Strasbourg, IGBMC UMR 7104, Illkirch, France; CNRS, UMR 7104, Illkirch, France; Inserm, UMR-S 1258, Illkirch, France; IGBMC, Institut de Génétique et de Biologie Moléculaire et Cellulaire, Illkirch, France. Electronic address:
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Department of Oral Immunology and Infectious Diseases, University of Louisville School of Dentistry, Louisville, KY, United States. Electronic address:
Retinoic acid (RA) signaling plays multiple essential roles in development of the head and face. Animal models with mutations in genes involved in RA signaling have enabled understanding of craniofacial morphogenic processes that are regulated by the retinoid pathway. During craniofacial morphogenesis RA signaling is active in spatially restricted domains defined by the expression of genes involved in RA production and RA breakdown.
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Department of Pharmacology, School of Medicine, Case Western Reserve University, Cleveland, OH, United States. Electronic address:
Animals perceiving light through visual pigments have evolved pathways for absorbing, transporting, and metabolizing the precursors essential for synthesis of their retinylidene chromophores. Over the past decades, our understanding of this metabolism has grown significantly. Through genetic manipulation, researchers gained insights into the metabolic complexity of the pathways mediating the flow of chromophore precursors throughout the body, and their enrichment within the eyes.
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University of Michigan, Department of Pharmacology, Caswell Diabetes Institute, Ann Arbor, MI, United States. Electronic address:
All-trans retinoic acid (ATRA) signaling is essential in numerous different biological contexts. This review highlights the diverse roles of ATRA during development, function, and diseases of the pancreas. ATRA is essential to specify pancreatic progenitors from gut tube endoderm, endocrine and exocrine differentiation, and adult islet function.
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