Global characteristics of childhood acute promyelocytic leukemia

Abstract

Acute promyelocytic leukemia (APL) comprises approximately 5–10% of childhood acute myeloid leukemia (AML) cases in the US. While variation in this percentage among other populations was noted previously, global patterns of childhood APL have not been thoroughly characterized. In this comprehensive review of childhood APL, we examined its geographic pattern and the potential contribution of environmental factors to observed variation. In 142 studies (spanning >60 countries) identified, variation was apparent—de novo APL represented from 2% (Switzerland) to >50% (Nicaragua) of childhood AML in different geographic regions. Because a limited number of previous studies addressed specific environmental exposures that potentially underlie childhood APL development, we gathered 28 childhood cases of therapy-related APL, which exemplified associations between prior exposures to chemotherapeutic drugs/radiation and APL diagnosis. Future population-based studies examining childhood APL patterns and the potential association with specific environmental exposures and other risk factors are needed.

Treatment Outcome and the Genetic Characteristics of Acute Promyelocytic Leukemia in Children in Poland From 2005 to 2018

“…Acute promyelocytic leukemia (APL) is a specific subtype of acute myeloid leukemia (AML). In most cases, it is characterized by translocation (15;17) with the PML-RARA fusion gene (1)(2)(3), but about 1-2% of APL cases are due to rare variant translocations including ZBTB16/RARA, NMP/RARA, NUMA/RARA, STAT5B/RARA, PRKAR1a/RARA, BCOR/RARA, and FIP1L1/RARA (4)(5)(6). APL comprises about 5-10% of pediatric AML (1) and about 0.4% of all malignancies in children.…”

“…Methods of the treatment used in children are based on clinical studies performed on greater adult population. Until the late 1980's, APL was the most lethal subtype of AML (2,3). The result of the treatment improved significantly since the 1980's when specific treatment with all-trans retinoic acid (ATRA) was introduced (2, 3, 7-9).…”

“…Use of the specific treatment in APL allowed reduction of the chemotherapy especially cumulative anthracycline doses (13,(15)(16)(17). The main causes of the treatment failure are still early deaths, mostly in the course of intracranial hemorrhage (2,3,18). In the large analysis comprising 683 patients from different international studies, initial high WBC counts and obesity were found as likely predictors of thrombohemorrhagic early deaths in childhood APL (18).…”

Association of Matrix Metallopeptidase-2 Promoter Polymorphisms With the Risk of Childhood Leukemia

“…According to epidemiology, leukemia is the most common type of childhood cancer (1,2), and accounts for 25-35% of cases of childhood cancer among most populations investigated (1,3). There are two major subtypes of childhood leukemia, acute myeloid leukemia (AML) and acute lymphocytic leukemia (ALL), with the former accounting for about 76% of childhood leukemia cases (4,5).…”

A Paediatric Acute Promyelocytic Leukaemia Patient Harbouring a Cryptic <b><i>PML-RARA</i></b> Insertion due to a Complex Structural Chromosome 17 Rearrangement

“…APL is considered to be rare in children [Ravandi et al, 2012], but its frequency varies among childhood AML across the world, the lowest found in Switzerland (2.4%) and the highest in Nicaragua (58.8%). In Australia, its frequency is 17.8% of childhood AML [Zhang et al, 2015].…”