Adèle de Masson

Severe Aplastic Anemia Associated With Eosinophilic Fasciitis: Report of 4 Cases and Review of the Literature

AbstractDiffuse eosinophilic fasciitis (Shulman disease) is a rare sclerodermiform syndrome that, in most cases, resolves spontaneously or after corticosteroid therapy. It has been associated with hematologic disorders, such as aplastic anemia. The clinical features and long-term outcomes of patients with eosinophilic fasciitis and associated aplastic anemia have been poorly described. We report the cases of 4 patients with eosinophilic fasciitis and associated severe aplastic anemia. For 3 of these patients, aplastic anemia was refractory to conventional immunosuppressive therapy with antithymocyte globulin and cyclosporine. One of the patients received rituximab as a second-line therapy with significant efficacy for both the skin and hematologic symptoms. To our knowledge, this report is the first to describe rituximab used to treat eosinophilic fasciitis with associated aplastic anemia.In a literature review, we identified 19 additional cases of eosinophilic fasciitis and aplastic anemia. Compared to patients with isolated eosinophilic fasciitis, patients with eosinophilic fasciitis and associated aplastic anemia were more likely to be men (70%) and older (mean age, 56 yr; range, 18–71 yr). Corticosteroid-containing regimens improved skin symptoms in 5 (42%) of 12 cases but were ineffective in the treatment of associated aplastic anemia in all but 1 case. Aplastic anemia was profound in 13 cases (57%) and was the cause of death in 8 cases (35%). Only 5 patients (22%) achieved long-term remission (allogeneic hematopoietic stem cell transplantation: n = 2; cyclosporine-containing regimen: n = 2; high-dose corticosteroid-based regimen: n = 1).

APRIL levels are associated with disease activity in human chronic graft-versus-host disease

After allogeneic hematopoietic stem cell transplantation (AHSCT), acute GVHD is almost entirely mediated by donor T cells whereas chronic graft- versus -host disease (cGVHD) also involves donor B cells.[1][1] A breakdown in peripheral B cell homeostasis in cGVHD is in part due to high levels of B

Purification and Immunophenotypic Characterization of Human B Cells with Regulatory Functions

The analysis of human B cell populations of the blood relies on the expression of surface markers, mainly CD19, CD24, CD38, and CD27. According to these surface markers, three main B cell subsets can be identified in the blood: immature transitional B cells (CD19(+)CD24(high)CD38(high)), naïve B cells (CD19(+)CD24(int)CD38(int)) that have not encountered an antigen, and memory B cells (CD19(+)CD27(+)). To date, human B cells with regulatory functions have been essentially described within the CD24(high)CD38(high) transitional B cell subset. CD24(high)CD38(high) transitional B cells are able to produce interleukin 10 (IL-10) and to regulate in vitro Th1 and Th17 CD4(+) T cell activation. Here, we provide the methods to analyze and purify the CD24(high)CD38(high) transitional B cell subset for further in vitro experiments. We also provide a reliable method to detect B cell IL-10 production using intracellular cytokine staining.

Dermatopulmonary Syndrome Associated With Anti-MDA5 Antibodies After Allogeneic Hematopoietic Stem Cell Transplantation

Importance Chronic graft-vs-host-disease (cGVHD) after allogeneic stem cell transplantation (AHSCT) may resemble autoimmune diseases. Anti-MDA5 (melanoma differentiation–associated gene 5) dermatopulmonary syndrome is a subset of dermatomyositis defined by specific clinical features and detection of anti-MDA5-antibodies in the serum. Objective To characterize the clinical features of patients who underwent AHSCT and screened positively for anti-MDA5 antibodies. Design, Setting, and Participants For this monocentric retrospective study, we exained 81 patients screened for anti-MDA5 antibodies at a specific dermatological or pulmonary postallograft consultation between January 2014 to September 2015 at a National Reference Center; 2 additional patients not seen at this consultation but having clinical features suggestive of anti-MDA5 syndrome were included. Twenty serum samples from patients after AHSCT without cGVHD were used as controls. Main Outcomes and Measures Anti-MDA5 antibodies screened using an immunodot assay. Results Of 83 patients who underwent AHSCT (mean [SD] age, 47 [14] years), 6 patients tested positive for anti-MDA5 antibodies (mean [SD] age, 43 [16] years) including 4 patients with interstitial lung disease and 3 patients with cutaneous clinical features similar to anti-MDA5 skin symptoms encountered in patients who have not undergone AHSCT, namely finger pad inflammation, palmar violaceous papules, and digital ulcerations. Three patients had severe respiratory symptoms resistant to systemic steroids, and 1 patient died of severe interstitial lung disease. Conclusions and Relevance The clinical features and long-term prognosis of patients who underwent AHSCT and test positively for anti-MDA5 antibodies should be evaluated in large prospective studies.

EF/SSc overlap syndrome and aplastic anaemia resistant to immunosuppressive therapy

Thomas Hügle, Andreas Bircher and Ulrich A. Walker Department of Rheumatology, University of Basel, FelixPlatter-Spital and Department of Dermatology Allergy Unit, University Hospital Basel, Basel, Switzerland. Accepted 5 October 2011 Correspondence to: Ulrich A. Walker, Department of Rheumatology, University of Basel, Burgfelderstr. 101, CH 4012, Basel, Switzerland. E-mail: ulrich.walker@ fps-basel.ch

Tumor necrosis factor-alpha inhibitors for the treatment of pyoderma gangrenosum not associated with inflammatory bowel diseases: a multicenter retrospective study

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