[Acquired, non-amyloid related factor X deficiency: A first case associated with atypical chronic lymphocytic leukemia and literature review]. / Déficit non amyloïde acquis en facteur X : un premier cas satellite d'une leucémie lymphoïde chronique atypique et revue de la littérature.

INTRODUCTION: Acquired factor X deficiency is in most cases associated with AL amyloidosis. Acquired non-amyloid related factor X deficiency (DNAA-FX) has been exceptionally reported in the literature. CASE REPORT: We report the first case of acquired, non-amyloid related factor X deficiency associated with atypical chronic lymphoid leukemia in a 66-year-old patient with spontaneous hematomas. After therapeutic failure with polyclonal intravenous immunoglobulins, specific lymphoid malignancy treatment allowed symptoms and coagulation disorder resolution. CONCLUSION: DNAA-FX should be considered in case of bleeding events or coagulation disorders during low-grade hematological malignancies. Its occurrence can be considered as a treatment indication to prevent potentially fatal bleeding complications.

Highly favorable outcome in BRCA-mutated metastatic breast cancer patients receiving high-dose chemotherapy and autologous hematopoietic stem cell transplantation.

Breast cancer carrying BRCA mutation may be highly sensitive to DNA-damaging agents. We hypothesized a better outcome for BRCA-mutated (BRCA(mut)) metastatic breast cancer (MBC) patients receiving high-dose chemotherapy and autologous hematopoietic stem cell transplantation (HDC AHSCT) versus unaffected BRCA (BRCA wild type; (BRCA(wt))) or patients without documented BRCA mutation (BRCA untested (BRCA(ut))). All female patients treated for MBC with AHSCT at Institut Paoli-Calmettes between 2003 and 2012 were included. BRCA(mut) and BRCA(wt) patients were identified from our institutional genetic database. Overall survival (OS) was the primary end point. A total of 235 patients were included. In all, 15 patients were BRCA(mut), 62 BRCA(wt) and 149 BRCA(ut). In multivariate analyses, the BRCA(mut) status was an independent prognostic factor for OS (hazard ratio (HR): 3.08, 95% confidence interval (CI): 1.10-8.64, P=0.0326) and PFS (HR: 2.52, 95% CI :1.29-4.91, P=0.0069). In this large series of MBC receiving HDC AHSCT, we report a highly favorable survival outcome in the subset of patients with documented germline BRCA mutations.

[Bing-Neel syndrome: Report of 4 cases and literature review]. / Syndrome de Bing et Neel : à propos de 4 cas et revue de la littérature.

INTRODUCTION: Neurological manifestations during Waldenstrom disease are common and are usually related to immune peripheral neuropathy or serum hyperviscosity syndrome. The infiltration of the central nervous system by the lymphoproliferative syndrome is known as the Bing-Neel syndrome. This extremely rare entity remains poorly described in the literature. CASE REPORTS: We report on 4 cases of patients for whom central neurological disorders led to the diagnosis of a Bing and Neel syndrome. These four cases illustrate different clinical presentations, diagnosis, therapeutic options, and outcome in this syndrome. Based on our literature review, we discuss about these differences. CONCLUSION: The polymorphic clinical manifestations of Bing and Neel syndrome can mimic many diagnoses. However, it may be necessary to consider this diagnosis. Cerebrospinal fluid analysis and MRI may allow rapid diagnosis or guide a biopsy. Prolonged remissions are possible with appropriate treatment.

[Can we prevent or cure infection-related cancers?]. / Peut-on prévenir ou guérir les cancers d'origine infectieuse?

Infections are an important cause of cancer in the world, representing approximately 16% of the neoplasia. Ten infectious agents have been classified as carcinogens of group I. Four of these pathogens (Helicobacter pylori, hepatitis B and C viruses, and some human papillomavirus) are responsible for 95% of cases of infection-related cancers. The carcinogenesis mechanisms are multiple, either direct via certain proteins from these microorganisms, or more often indirect through chronic inflammation. This allowed to consider prevention of certain cancers, for example with a prophylactic vaccine strategy. Advances were also made in the curative field. However, efforts remain to be done to discover new infectious causes of cancer and refine the understanding of the mechanisms of carcinogenesis, for a better targeting of anticancer therapeutics.