Survival of European patients diagnosed with myeloid malignancies: a HAEMACARE study

Abstract

Population-based information on the survival of patients with myeloid malignancies is rare mainly because some entities were not recognized as malignant until the publication of the third revision of the International Classification of Diseases for Oncology and World Health Organization classification in 2000. In this study we report the survival of patients with myeloid malignancies, classified by updated criteria, in Europe. We analyzed 58,800 cases incident between 1995 to 2002 in 48 population-based cancer registries from 20 European countries, classified into HAEMACARE myeloid malignancy groupings. The period approach was used to estimate 5-year relative survival in 2000-2002. The relative overall survival rate was 37%, but varied significantly between the major groups: being 17% for acute myeloid leukemia, 20% for myelodysplastic/myeloproliferative neoplasms, 31% for myelodysplastic syndromes and 63% for myeloproliferative neoplasms. Survival of patients with individual disease entities ranged from 90% for those with essential thrombocythemia to 4% for those with acute myeloid leukemia with multilineage dysplasia. Regional European variations in survival were conspicuous for myeloproliferative neoplasms, with survival rates being lowest in Eastern Europe. This is the first paper to present large-scale, European survival data for patients with myeloid malignancies using prognosis-based groupings of entities defined by the third revision of the International Classification of Diseases for Oncology/World Health Organization classifications. Poor survival in some parts of Europe, particularly for treatable diseases such as chronic myeloid leukemia, is of concern for hematologists and public health authorities.

Figure 1.

(A) Period estimates of relative survival in 2000-2002 by years from diagnosis for patients with acute myeloid leukemia (AML), AML other, acute promyelocytic leukemia (APL), and AML with multilineage dysplasia. (B) Period estimates of relative survival in 2000-2002 by years from diagnosis for patients with myeloproliferative neoplasms (MPN), chronic myeloid leukemia (CML), chronic myeloproliferative neoplasms NOS (chronic MPD, NOS), polycythemia vera (PV), myelosclerosis with myeloid metaplasia (MSMM), and essential thrombocythemia (ET). (C) Period estimates of relative survival in 2000-2002 by years from diagnosis for patients with myelodysplastic syndrome (MDS), myelodysplastic syndrome NOS (MDS, NOS), refractory anemia, refractory anemia with ring sideroblasts, and refractory anemia with excess blasts.

Figure 2.

Age-adjusted period estimates in 2000-2002 of 5-year relative survival for patients with acute myeloid leukemia (AML), acute promyelocytic leukemia (APL), myeloproliferative neoplasm (MPN) and chronic myeloid leukemia (CML), by European region.