Twenty-five years of epidemiological recording on myeloid malignancies: data from the specialized registry of hematologic malignancies of Cote d'Or (Burgundy, France).

BACKGROUND: Epidemiological data on myeloid malignancies are very rare in the literature due to a lack of registration by cancer registries until 2000. The Registry of Hematologic Malignancies of the Côte d'Or Department in France has, however, steadfastly registered data on cases occurring in the Department since 1980, resulting, to date, in a database of over 5,000 cases classified according to the ICD-O-3 classification, following the most recent World Health Organization classification criteria. DESIGN AND METHODS: Twenty-five years of data on myeloid malignancies, including acute myeloid leukemia, myeloproliferative neoplasms, myelodysplastic syndromes and myelodysplastic/myeloproliferative syndromes were analyzed. World population standardized incidence rates were calculated as were as observed and relative survival. RESULTS: Incidence rates per 100,000 inhabitants/year were 2.5 for acute myeloid leukemia, 1.3 for myelodysplastic syndromes, 3.2 for myeloproliferative neoplasms and 0.6 for myelodysplastic/myeloproliferative syndromes. It was found that the incidence rate of myelodysplastic syndromes increased significantly over the period. The median overall survival is 8.9 months for patients with acute myeloid leukemia, 33.8 months for patients with myelodysplastic syndromes, 91.7 months for those with myeloproliferative neoplasms and 26.6 months for patients with myelodysplastic/myeloproliferative syndromes. Observed and relative 20-year survival rates are, respectively, 12% and 13% in acute myeloid leukemia, 2% and 6% in myelodysplastic syndromes and 20% and 34% in myeloproliferative neoplasms. CONCLUSIONS: These population-based data on myeloid malignancies are the first data collected over such a long period and provide interesting information for clinicians and public health authorities, particularly given the paucity of other long-term, population-based data from cancer registries.

Significant increase in the apparent incidence of essential thrombocythemia related to new WHO diagnostic criteria: a population-based study.

To observe the effect of the new World Health Organization (WHO) criteria on the incidence of myeloproliferative neoplasms, we performed a retrospective study of a population-based registry in the Côte d'Or area, France, from 1980 to 2007. A total of 524 myeloproliferative neoplasms were registered for the 1980-2007 period, including 135 polycythemia vera, 308 essential thrombocythemia and 81 idiopathic myelofibroses. No change in the incidence of either polycythemia vera or idiopathic myelofibrosis was observed for the 2005-2007 period, compared to 1980-2004. On the contrary, a pronounced increase in the incidence of essential thrombocythemia was noted after 2005, mainly due to the use of JAK2 mutation screening and a lower threshold of platelet count. Our study confirms the relevance of the new WHO diagnostic criteria in allowing earlier diagnosis of essential thrombocythemia.

AML transformation in 56 patients with Ph- MPD in two well defined populations.

The Philadelphia chromosome-negative (Ph-) chronic myeloproliferative disorders (MPD) have an inherent tendency for transformation into acute myelogenous leukaemia (AML). The long-term rate of leukaemic transformation in unselected MPD patients was studied in well-defined MPD populations in Gothenburg, Sweden and the Côte d'Or area, Burgundy, France, respectively. Over a median observation time of 15 yr, 56 subjects (7%) out of a total of 795 patients with Ph- MPD transformed to AML. The yearly incidence of AML transformation was 0.38% in polycythaemia vera (PV), 0.37% in essential thrombocythaemia (ET) and 1.09% in idiopathic myelofibrosis (IMF). The incidence of AML development was significantly higher in IMF as compared with both PV and ET (P = 0.002 and P = 0.02, respectively). Six of the patients who developed AML had never been treated with cytoreductive agents and two had only been exposed to interferon. In IMF, the average time from diagnosis to AML transformation was 42 +/- 33 months, which was significantly shorter than for both PV and ET (88 +/- 56 and 76 +/- 57 months; P = 0.0075 and P = 0.027, respectively). The time from diagnosis to AML transformation appears to be a continuous event as regards all three MPD entities. It was shown that 17 out of the 18 patients with PV who developed AML were females; this was true despite the fact that the male/female ratio for the whole PV group was 146/171 (0.85). As regards ET and IMF patients who transformed to AML, the gender ratio showed slight male predominance (1.33 and 1.13, respectively). The average survival time for the 56 MPD patients who developed AML was 4.6 +/- 5.5 (range 0-28) months and did not differ with respect to the three subtypes of pre-AML MPD.

Case-control study of lymphoid neoplasm in three French areas: description, alcohol and tobacco consumption.

A multi-centre hospital-based case-control study was conducted in three regions of France between 2000 and 2003 in order to establish the risk factors of lymphoid neoplasms. We report here results concerning alcohol and tobacco consumption. A total of 298 cases and 276 controls, case-matched by inclusion centre, age and sex were included. Cases were classified according to the World Health Organization classification and validated by an expert panel of eight pathologists. Overall alcohol intake did not incur any risk increase for non-Hodgkin's lymphoma. Wine consumption marginally increased the risk of follicular lymphoma [odds ratio=2.19 (0.83-5.80)], with a higher risk for drinkers who started before the age of 20 years [odds ratio=4.04 (1.19-13.76)] and for drinkers who consumed more than 19 g of alcohol per day [odds ratio=4.37 (1.04-18.45)]. Beer and spirit consumption was not linked to non-Hodgkin's lymphoma risk. Tobacco consumption did not show a risk increase. The risk increase of follicular lymphoma due to wine consumption was new. We discuss this risk based on the French context, France being the European country with the highest alcohol consumption, particularly of wine.

[Epidemiology and etiology of non-Hodgkin's lymphoma]. / Epidémiologie et étiologie des lymphomes non hodgkiniens.

Nowadays, non-hodgkin lymphoma (NHL) presents the fifth highest cancer incidence rate. It is one of the few cancers in which incidence has been increasing over the last decades in the world. Even though therapeutic progress allow a favourable evolution in one case out of two, NHL remains preoccupying, especially because of the lack of knowledge regarding its aetiology. Indeed, several factors have been studied and may play a concomitant role: factors linked to genetics, immunodepression, viruses, professional or non-professional toxics. We will review results from different studies evaluating the role of those factors after having reported current knowledge on NHL.

Clinical value of the quantitative expression of the three epitopes of CD34 in 300 cases of acute myeloid leukemia.

BACKGROUND AND OBJECTIVES: The various epitopes of the CD34 molecule have been classified according to their different sensitivities to enzymatic cleavage by neuraminidase, chymopapain and a glycoprotease from Pasteurella haemolytica. Although monoclonal antibodies have been developed that specifically identify these epitopes, few studies have evaluated the distribution and quantitative expression of such epitopes on leukemic blasts. DESIGN AND METHODS: We report here a prospective multicenter study in which we examined and quantified the expression of the 3 classes of CD34 on fresh leukemic blast cells from 300 cases of acute myeloid leukemia (AML). The binding of monoclonal antibodies was studied by flow cytometry, allowing evaluation of blast cell positivity as well as their mean fluorescence intensity. These quantitative data were made comparable between centers by means of a calibration curve established with the same reagents in all laboratories. RESULTS: Quantitative expression of class I epitope was significantly higher than that of class II and class III epitopes (p<0.0001). The three classes were more frequently expressed in M0 and M1 and less in M3 and M5. The highest levels of CD34 expression were observed in M2, M0 and M1 and the lowest in M3, M5 and BAL for class II and III. CD34 expression was lower for all classes in cases with a normal karyotype, compared to in cases with structural or numerical abnormalities. INTERPRETATION AND CONCLUSIONS: In cases with a t(9;22) the expression of class I was significantly higher than that of class II and III and the opposite was observed in AML with t(15;17). Moreover, as a whole, a high intensity of class III CD34 appeared to be a marker of good prognosis.