Childhood Discoid Lupus in Identical Twins

Abstract: We report the exceptional case of 11‐year‐old identical male twins who both developed discoid lupus erythematosus lesions. Although systemic lupus erythematosus has often been reported in identical twins, discoid lupus erythematosus has only ocassionaly been described, with only one other case in twin children, as far as we are aware.     

Salivary gland dysfunction (‘dry mouth’) in patients with cancer: a consensus statement

DAVIES A., BAGG J., LAVERTY D., SWEENEY P., FILBET M., NEWBOLD K., DE ANDRÉS J. & MERCADANTE S. (2010) European Journal of Cancer Care 19 , 172–177
Salivary gland dysfunction (‘dry mouth’) in patients with cancer: a consensus statement A group of interested professionals was convened to develop some evidence‐based recommendations on the management of salivary gland dysfunction (SGD) in oncology patients. A Medline search was performed to identify the literature on SGD. The abstracts of all identified papers were read, and the full texts of all relevant papers were reviewed. The evidence was graded according to the Scottish Intercollegiate Guidelines Network grading system for recommendations in evidence‐based guidelines. The summary of the main recommendations are: (1) patients with cancer should be regularly assessed for SGD (grade of recommendation – D); (2) the management of SGD should be individualised (D); (3) consideration should be given to strategies to prevent the development of radiation‐induced SGD (C); (4) consideration should be given to treatment of the cause(s) of the SGD (C); (5) the treatment of choice for the symptomatic management of SGD is use of an appropriate saliva stimulant (C); (6) consideration should be given to prevention of the complications of the SGD (D); (7) consideration should be given to treatment of the complications of the SGD (D); and (8) patients with SGD should be regularly reassessed (D).     

Bortezomib delays the onset of factor VIII inhibitors in experimental hemophilia A,but fails to eliminate established anti‐factor VIII IgG‐producing cells

Summary. Background: Replacement therapy with exogenous factor VIII to treat hemorrhages induces inhibitory anti‐FVIII antibodies in up to 30% of patients with hemophilia A. Current approaches to eradicate FVIII inhibitors using high‐dose FVIII injection protocols (immune tolerance induction) or anti‐CD20 depleting antibodies (Rituximab) demonstrate limited efficacy; they are extremely expensive and/or require stringent compliance from the patients. Objectives: To investigate whether the proteasome inhibitor bortezomib, which depletes plasmocytes, modulates the anti‐FVIII immune response in FVIII‐deficient mice. Methods and results: Preventive 4‐week treatment of naïve mice with bortezomib at the time of FVIII administration delayed the development of inhibitory anti‐FVIII IgG, and depleted plasma cells as well as different lymphoid cell subsets. Conversely, curative treatment of inhibitor‐positive mice for 10 weeks, along with FVIII administration, failed to eradicate FVIII inhibitors to extents that would be clinically relevant if achieved in patients. Accordingly, bortezomib did not eradicate anti‐FVIII IgG‐secreting plasmocytes that had homed to survival niches in the bone marrow, despite significant elimination of total plasma cells. Conclusions: The data suggest that strategies for the efficient reduction of anti‐FVIII IgG titers in patients with hemophilia A should rely on competition for survival niches for plasmocytes in the bone marrow rather than the mere use of proteasome inhibitors.