Systematic Literature Review of the Global Incidence and Prevalence of Myelodysplastic Syndrome and Acute Myeloid Leukemia
Authors: Lubeck Deborah P , Danese Mark , Jennifer Duryea , Miller Kimberly , Richhariya Akshara , Garfin Phillip M. 
Published: November 2019

Abstract
Introduction: Acute myeloid leukemia (AML) consists of a group of relatively well-defined hematopoietic neoplasms, while myelodysplastic syndrome (MDS) comprises a heterogeneous group of hematopoietic stem cell disorders. These disorders may occur de novo or may arise secondary to prior malignancies for which patients received cytotoxic chemotherapy and/or radiation therapy. While AML and MDS are reported in a number of national cancer registries, competing classifications prior to 2001 often preclude comparison of results globally. We conducted a systematic literature review in order to identify global rates of the incidence and prevalence of AML and MDS.
Methods: We searched Embase and MEDLINE for published studies and recent conference abstracts on the incidence and/or prevalence of AML and MDS for data collected from 2001-2016. This time period parallels the introduction of the World Health Organization (WHO) classification of hematopoietic neoplasms. Two reviewers independently screened all abstracts and selected articles, and subsequently abstracted multiple data elements including: AML or MDS, geographic region, standardized incidence and/or prevalence rates, years of data collection, disease classification criteria (e.g. WHO, French American British (FAB), ICD-O-3, ICD-9/10), if condition was diagnosed de novo or treatment associated, age category, and gender. Incidence rates and prevalence by sub-disease classification were also abstracted when available.
Results: Literature Review: Our search strategy yielded 874 abstracts initially reviewed for applicability. Of these, 84 were selected for article screening; 44 articles were excluded after screening and 40 articles proceeded to data abstraction and were included in analyses. 25 studies reported on MDS incidence and 2 on MDS prevalence; 17 studies reported on AML incidence and 4 on AML prevalence (some studies report both AML and MDS and incidence and prevalence). While the majority of studies are based on existing regional disease registries, a number describe analyses based on administrative claims data, or patient charts. Nine (9) studies reported on therapy associated incidence. Classification of AML or MDS was based on FAB (9 studies), WHO (10 studies), ICD-O-3 (11 studies) or ICD-9/10 (7 studies) criteria. Three studies were classified on the basis of chart review and/or clinical diagnosis by a physician. Regional distribution was: North America (10 studies), Europe (17 studies), Australia (4 studies), and the Rest of World (including 3 studies in Asia, 2 studies in Africa, 2 in South America and 2 in the Middle East).
Analysis: Incidence rates are reported in Figure 1 by region for those studies reporting overall rates across clinical factors, age and gender (27 articles). Incidence of AML as a result of treatment for another cancer ranged from 0.06-2.6 per 100,000 and for MDS from 0.06-0.26 per 100,000. In general, incidence rates increased with increasing age in studies that reported results by age group. Prevalence of AML ranged from 0.6-11.0 per 100,000 and for MDS ranged from 0.22-13.2 per 100,000 for all age categories, genders and ethnicities.
Discussion:This is the first study to report on the global incidence of AML and MDS and whether or not the disease occurs as a result of treatment for another oncologic condition or occurs de novo. Variation in study designs and heterogeneous population characteristics make interpretation of results challenging.
Figure 1 Overall Incidence Rates for AML and MDS by Geographic Region Figure 1. Overall Incidence Rates for AML and MDS by Geographic Region

Disclosures
Lubeck: Outcomes Insights: Employment. Danese:Outcomes Insights: Employment. Jennifer:Outcomes Insights: Employment. Miller:Outcomes Insights: Consultancy. Richhariya:Seattle Genetics, Inc.: Employment. Garfin:Seattle Genetics, Inc.: Employment.