Clin Pediatr Hematol Oncol.  2015 Apr;22(1):8-14. 10.15264/cpho.2015.22.1.8.

Diagnosis and Treatment of Pediatric Acute Myeloid Leukemia

• Lee JW
• Cho B

• KMID: 2168914
• DOI: http://doi.org/10.15264/cpho.2015.22.1.8

Abstract

Acute myeloid leukemia (AML) is a heterogeneous malignancy that comprises 25-30% of pediatric leukemias in Korea. Several inherited diseases, such as Down syndrome and Fanconi anemia, predispose towards AML leukemogenesis. Subgrouping of AML is a key diagnostic step, previously done with the French-American-British (FAB) classification and recently complemented by that of the World Health Organization (WHO). An important feature of AML is the possibility of chloroma at diagnosis, which, if detected, requires follow-up evaluation to determine treatment response. Numerous genetic abnormalities with prognostic relevance have recently been found, the most important of which include those of the core-binding factor (CBF) leukemias, and FLT3-ITD mutation. These genetic abnormalities, combined with patient response to initial treatment, allow for a scheme of risk stratification, and the current consensus is to treat low risk patients with chemotherapy only, whereas high risk patients may receive allogeneic transplant in first remission, although the benefits of transplant remain inconclusive. Overall, the outcome of children and adolescents with AML has improved significantly so that many clinical trials now report event-free survival of around 60%. However, much of this improvement stems from better supportive care and transplant methods, and the genetics-based diagnostic advances in AML have yet to result in enhanced treatment. New therapeutics, including possibly targeted therapy, are necessary to further improve the outcome of pediatric AML.

Keyword

Acute myeloid leukemia; Children; Genetic abnormalities; Chemotherapy; Hematopoietic cell transplantation

MeSH Terms

Adolescent
Child
Classification
Complement System Proteins
Consensus
Core Binding Factors
Diagnosis*
Disease-Free Survival
Down Syndrome
Drug Therapy
Fanconi Anemia
Humans
Korea
Leukemia
Leukemia, Myeloid, Acute*
Sarcoma, Myeloid
World Health Organization
Complement System Proteins
Core Binding Factors