Mantle cell lymphoma epidemiology: a population-based study in France

Only limited population-based data are available on mantle cell lymphoma (MCL), a relatively rare and aggressive mature B cell non-Hodgkin lymphoma (NHL) entity. We conducted an epidemiological study based on the three French registries devoted to haematological malignancies over the period 2002–2006. Main clinical features and management characteristics were collected. Incidence and survival rates were estimated, and independent prognostic factors were analysed. MCL was diagnosed in 135 patients between 2002 and 2006. Seventy-four percent of patients were men. Age-standardised incidence rate of MCL (per 100,000) was 1.1 in men and 0.26 in women. Median age at diagnosis was 72 years (range 30–92). Advanced-stage (III or IV) disease was diagnosed in 81.5 % of patients, and 55 % of them were identified as high risk according to MCL International Prognostic Index (MIPI). Median net survival time was 41 months (95 % confidence interval (CI), 38–62). Main independent prognostic factors were age at diagnosis, performance status and use of rituximab in first-line treatment. Median overall survival was 36 months (95 % CI, 27–40) for high MIPI and 60 months (95 % CI, 35–74) for low/intermediate MIPI patients (p?=?0.02). This study confirms that MCL remains an aggressive NHL with a median overall survival less than 4 years and demonstrates that the use of rituximab has modified overall survival duration.     

Epidemiology,clinical picture and long-term outcomes of FIP1L1-PDGFRA-positive myeloid neoplasm with eosinophilia: Data from 151 patients

FIP1L1-PDGFRA-positive myeloid neoplasm with eosinophilia (F/P+ MN-eo) is a rare disease: robust epidemiological data are lacking and reported issues are scarce, of low sample-size and limited follow-up. Imatinib mesylate (IM) is highly efficient but no predictive factor of relapse after discontinuation has yet been identified. One hundred and fifty-one patients with F/P+ MN-eo (143 males; mean age at diagnosis 49 years; mean annual incidence: 0.18 case per million population) were included in this retrospective nationwide study involving all French laboratories who perform the search of F/P fusion gene (study period: 2003-2019). The main organs involved included the spleen (44%), skin (32%), lungs (30%), heart (19%) and central nervous system (9%). Serum vitamin B12 and tryptase levels were elevated in 74/79 (94%) and 45/57 (79%) patients, respectively, and none of the 31 patients initially treated with corticosteroids achieved complete hematologic remission. All 148 (98%) IM-treated patients achieved complete hematologic and molecular (when tested, n = 84) responses. Forty-six patients eventually discontinued IM, among whom 20 (57%) relapsed. In multivariate analysis, time to IM initiation (continuous HR: 1,01 [0.99-1,03]; P = .05) and duration of IM treatment (continuous HR: 0,97 [0,95-0,99]; P = .004) were independent factors of relapse after discontinuation of IM. After a mean follow-up of 80 (56) months, the 1, 5- and 10-year overall survival rates in IM-treated patients were 99%, 95% and 84% respectively. In F/P+ MN-eo, prompt initiation of IM and longer treatment durations may prevent relapses after discontinuation of IM.     

Amniotic band syndrome with limb amputation after exposure to mifepristone in early pregnancy

To date, the number of cases reported after exposure to mifepristone alone in early pregnancy is limited. In 24 cases, only 1 observation of fetal malformation associated with mifepristone has previously been reported. We report a case of amniotic band syndrome with limb amputation after exposure to mifepristone in early pregnancy. This association raises the question of a possible causal relationship.     

Is vaginal hysterectomy a risk factor for urinary incontinence at long-term follow-up?

OBJECTIVE: The purpose of this study was to evaluate the prevalence of urinary symptoms at long-term follow-up after vaginal hysterectomy. STUDY DESIGN: One hundred and seventeen patients, who had a vaginal hysterectomy for menorrhagia, from January 1991 to December 2001, answered to a self-report questionnaire about de novo urinary symptoms. The control group was a population of 116 patients who had a conservative treatment for dysfunctional uterine bleeding by endometrial thermocoagulation from January 1994 to December 2001. RESULTS: Patient characteristics (mean age, mean parity, menopausal status, smoking status, drink habits) were similar in the two groups. Mean follow-up was 4.6+/-2.2 years (range 1.5-11) after vaginal hysterectomy and 4+/-1.8 years (range 1.5-7) after conservative treatment. The prevalence of urinary symptoms, included urge and stress incontinence, were statistically similar in the two groups. CONCLUSION: This study reveals no risk of urge or stress urinary incontinence at long-term follow-up after vaginal hysterectomy, compared with conservative treatment.     

E-cadherin/beta-catenin/T-cell factor pathway is involved in smooth muscle cell proliferation elicited by oxidized low-density lipoprotein

The E-cadherin/beta-catenin/T-cell factor (Tcf) signaling pathway plays a crucial role in embryogenesis and carcinogenesis and has recently emerged in atherosclerosis. The aim of this work was to investigate whether this signaling pathway is involved in smooth muscle cell proliferation induced by oxidized low-density lipoprotein (LDL). In human aortic smooth muscle cells, mitogenic concentration of mildly oxidized LDL induced the activation of beta-catenin, as assessed by the dissociation of the beta-catenin/cadherin complex, and the concomitant rise of active beta-catenin in the cytosol. The oxidized LDL-induced rise of active beta-catenin required metalloproteinase activation, as well as epidermal growth factor receptor and Src signaling, as assessed by the use of pharmacological inhibitors and cells overexpressing a SrcK-inactive form. The concomitant phosphatidylinositol 3-kinase/Akt activation and glycogen synthase kinase 3-beta phosphorylation induced the inhibition of the proteasomal degradation of beta-catenin. Then active beta-catenin associated with Tcf4 and translocated into the nucleus. This enhanced the expression of the cell cycle activator cyclin D1. This crucial role of beta-catenin in the mitogenic effect of oxidized LDL was confirmed by silencing beta-catenin by specific small interfering RNA that blocked DNA synthesis. Immunohistochemistry staining of stable and disrupted plaques from carotid endarterectomy sections showed a correlation between active beta-catenin and Ki67, a proliferation marker, and a more intense staining in the smooth muscle cell layer surrounding the lipid core of disrupted plaques. In conclusion, the beta-catenin pathway is required for the mitogenic effect of oxidized LDL on human aortic smooth muscle cells. This study highlights the putative important role of the E-cadherin/beta-catenin/Tcf signaling pathway in atherosclerosis.     

Relationship between vascular endothelium and periodontal disease in atherosclerotic lesions: Review article

Inflammation and endothelial dysfunction are linked to the pathogenesis of atherosclerotic disease. Recent studies suggest that periodontal infection and the ensuing increase in the levels of inflammatory markers may be associated with myocardial infarction, peripheral vascular disease and cerebrovascular disease. The present article aimed at reviewing contemporary data on the pathophysiology of vascular endothelium and its association with periodontitis in the scenario of cardiovascular disease.     

MRI of endolymphatic hydrops in patients with vestibular schwannomas: a case-controlled study using non-enhanced T2-weighted images at 3 Teslas

European Archives of Oto-Rhino-Laryngology - Vestibular schwannomas (VS) may present with similar symptoms endolymphatic hydrops. Association between hydrops and internal auditory canal VS has been...