There are limited population-based studies of hairy cell leukemia (HCL), a rare chronic lymphoproliferative disorder of B-cells. We conducted a population-based study that included all patients diagnosed with HCL between 1996 and 2016 in Western Normandy. Recorded data focused on medical history, clinical presentation, biological results, treatment modalities in the first line and in relapsed/refractory patients and the occurrence of secondary malignancies. One hundred and twenty-three HCL patients were registered in the database. HCL represented 0.7% of all malignant hematological disorders and 3.0% of all leukemia. The overall age-standardized incidence ratio (SIR) was 0.39/100,000 inhabitants in men and 0.09/100,000 in women, and it remained stable over the 20-year period analyzed. One hundred and seven patients (88%) received first-line treatment, 33 patients (27%) received at least 2 lines of treatment and 14 patients (11%) received more than 2 lines. Cladribine used as first-line treatment induced a high hematological complete response (HCR) rate of 92%. The median overall survival (OS) was over 15 years, with 5-year and 10-year survival rates of 84% and 70.5%. No significant differences in OS were observed between men and women, between the calendar periods studied or between patients who received a single line treatment with IFN-α or PNA. The risk of relapse was higher with IFN-α treatment, requiring subsequent treatments in that patients. The time to next treatment (TTN) tends to be longer for PNAs compared to IFN-α even if difference is not significant. Secondary cancers were observed in 9/123 patients (7.3%) with solid tumors in 8 patients and hematological malignancy in one patient. Our data confirm in real life that single courses of cladribine administered to patients with HCL induce high response rates, the majority of which are HCR. Relapses seem less frequent than with IFN-α and the administration schedule is less restrictive for the patients. The emergence of chemo-immunotherapy and the development of effective new drugs such as recombinant immunotoxins and BRAF targeting will offer new possibilities in the management of HCL patients.

As the diagnostic criteria for chronic lymphocytic leukemia (CLL) and monoclonal B-lymphocytosis (MBL) have changed significantly over time, CLL and MBL epidemiology remains to be improved. We examined the incidence patterns and the epidemiology of CLL and MBL in a French registry.

Les etudes epidemiologiques descriptives sur les syndromes myelodysplasiques (SMD) sont peu nombreuses et montrent des taux bruts d’incidence variant entre 1,0/100 000 au Japon et 9,3/100 000 au Royaume-Uni. Nous presentons les donnees du Registre Regional des Hemopathies Malignes de Basse Normandie (RRHMBN) et nous montrons que les SMD representent 13 % de l’ensemble des hemopathies malignes. Les anemies refractaires (RA) sont les plus frequentes, representant pres de la moitie (48,4 %) de l’ensemble des SMD. Le taux d’incidence standardise (TSM) de l’ensemble des SMD sur la population mondiale est pour les trois departements de 2,55/100 000 ± 0,22 globalement, plus eleve chez l’homme (3,64 ± 0,38) que chez la femme (1,78 ± 0,25) (sex-ratio : 2,04). L’incidence des SMD augmente avec l’âge avec un taux specifique de 1,75/100 000 pour la tranche d’âge 50-54 ans et de 24,03 et 37,83/100 000 pour les tranches d’âge respectivement de 70-74 et 75-79 ans. Les variabilites d’incidence observees peuvent s’expliquer par la grande heterogeneite des SMD, les variations de classification des hemopathies malignes evoluant avec le temps et, enfin, l’absence de standardisation de l’enregistrement dans les differents registres. Les donnees d’un registre specialise sont necessaires pour ameliorer les connaissances de ces hemopathies malignes.

Les études épidémiologiques descriptives sur les syndromes myélodysplasiques (SMD) sont peu nombreuses et montrent des taux bruts d’incidence variant entre 1,0/100 000 au Japon et 9,3/100 000 au Royaume-Uni.