ABSTRACT
The treatment of chronic myeloid leukemia (CML) has seen several major advances over the past 30 years, notably with the introduction of interferon followed by Bcr-Abl tyrosine kinase inhibitors. We analyzed trends in the incidence of CML and patient survival in France. All cases recorded in five population-based registries between 1980 and 2009 were included. European (ESR) and world (WSR) standardized incidence rates as well as relative survival (RS) rates were estimated. We analyzed data for 781 patients (9863/3: 13.6%; 9875/3: 82.2%; 9876/3: 4.2%). ESR was 1.02 [95% confidence interval (CI) = 0.93-1.11] and WSR was 0.81 [95% CI = 0.72-0.90]. The five RS rates among patients with Philadelphia chromosome positive (Ph+) CML were 43.7% [30.9-61.9] when diagnosed in 1980-1986, 63.8% [56.9-71.5] in 1987-1999 and 88.7% [84.5-93.0] in 2000-2009. The 8-year RS rate of patients with Ph+ CML diagnosed in 2000-2009 was 83.3% [77.5-89.4]. Therapeutic innovations have thus led to a significant increase in long-term survival in the general CML patient-population.
Subject(s)
Leukemia, Myelogenous, Chronic, BCR-ABL Positive/epidemiology , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/therapy , Population Surveillance/methods , Registries/statistics & numerical data , Adolescent , Adult , Aged , Aged, 80 and over , Child , Child, Preschool , Disease Progression , Female , Follow-Up Studies , France/epidemiology , Humans , Incidence , Infant , Infant, Newborn , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/diagnosis , Male , Middle Aged , Survival Analysis , Young AdultABSTRACT
Multiple myeloma (MM) is the third most common haematologic malignancy in European countries, and is usually preceded by Monoclonal Gammopathy of Undetermined Significance (MGUS). Therefore epidemiologic studies of MGUS are very limited in a population-based status. Here we report all new cases of MGUS exhaustively recorded by the Basse-Normandie Regional Registry for Hematologic Malignancies (a French region registry) between January 1997 and December 2005, and analyze outcome of patients until 2009 in term of evolution in MM or death. All cases were analyzed by an expert file review, and MGUS diagnosis was retained for: evidence of a monoclonal component <30 g/l and no CRAB criteria (hyperCalcemia, renal insufficiency, anemia, bone lesions). We showed that the world standardized incidence rate (WSR) for MGUS was 3.76 ± 0.26 per 100,000 inhabitants, increasing regularly with age, and that the median overall survival (OS) was 115.9 months (CI 95%: 10.5-130.2 months) with 78.3% patients alive at 5 years (CI 95%: 74.1-81.9%). We also observed a rate of progression to multiple myeloma of 1.41% per year, concordant with previous reports in a reallife exhaustive registry.
