27 research outputs found

    Targeted therapy monitoring of BRAF-V600-mutant Erdheim-Chester disease by fast quantitative whole-body bone CZT-tomoscintigraphies

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    Abstract Erdheim-Chester disease (ECD) is a rare histiocytosis due to proto-oncogene mutations, primarily affecting the long bones and possibly being treated by novel targeted therapies. 18F-FDG PET is a reference technique for ECD assessment. However, we present a case where easier and more objective monitoring of the ECD-related bone metabolism abnormalities under treatment was obtained with the standardized uptake value-based information provided by fast whole-body [Tc-99 m]-HDP bone tomoscintigraphies (QWBT) recorded with a high-sensitivity CZT-camera/computed tomography (CT) hybrid system

    Antiphospholipid antibodies and the risk of thrombocytopenia in patients with systemic lupus erythematosus: A systematic review and meta-analysis

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    International audienceBackground: According to criteria for the classification of Systemic Lupus Erythematosus (SLE), thrombocytopenia is one of the disease-defining hematologic disorders. Since the recognition of Antiphospholipid Syndrome (APS), thrombocytopenia was frequently reported but several studies yielded contradictory results on the association between aPL-positivity and thrombocytopenia.Methods: We evaluated the role of antiphospholipid antibodies (aPL) and different aPL profiles on the risk of thrombocytopenia in SLE patients by conducting a systematic review and meta-analysis of available literature from 1987 to 2018. MEDLINE, EMBASE, Cochrane Library, congress abstracts, and reference lists of eligible studies were searched. Studies were selected if they included SLE patients with descriptions of the exposure to aPL and the outcomes (thrombocytopenia). Two reviewers extracted study characteristics and outcome data from published reports. Estimates were pooled using random effects models and sensitivity analyses. We followed the PRISMA guidelines for all stages of the meta-analysis. PROSPERO registration number: CRD42015027378.Results: From 3278 articles identified, 53 studies met inclusion criteria amounting to 9019 SLE patients. Twenty-nine percent of aPL-positive SLE patients had thrombocytopenia compared to 15.1% in aPL-negative SLE patients. The overall pooled Odds Ratio (OR) for thrombocytopenia in aPL positive patients was 2.48 (95% CI; 2.10-2.93). Among aPL subtypes, the risk of thrombocytopenia was highest for lupus anticoagulant (OR = 3.56 [95% CI, 2.57-5.25]), IgM anti-β2-GP1(OR = 2.87 [95% CI; 2.57-5.25]), IgG and IgM anticardiolipin antibodies (OR = 1.87 [95% CI; 1.52-2.31] and OR = 1.73 [95% CI; 1.36-2.19] respectively).Conclusions: The occurrence of thrombocytopenia was strongly determined by various aPL profiles in SLE patients. While the association between IgM antibodies and other APS manifestations including thrombosis is debated, IgM isotypes are helpful in the risk stratification of thrombocytopenia in SLE

    Radicals of Free and Zinc(II)-Coordinated α-Azophenols

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    The tridentate 2-tert-butyl-4-methoxy-6-(quinolin-8-ylazo)phenol ligand HL has been synthesized and structurally characterized. Its redox activity has been investigated by electrochemical measurements and DFT calculations. Oxidation of HL (irreversible process) affords primarily a hydrogen-bonded phenoxyl radical, whereas its reduction affords an iminosemiquinonate radical species. The reaction of two equivalents of HL with Zn(OAc)2 affords the zinc complex Zn(L)2, which has been structurally characterized. Its oxidation is easier than that of HL and is reversible. The EPR spectrum of [Zn(L)(L·)]+ shows an unresolved (S = 1/2) signal at g = 2.005, while [Zn(L·)]2+ exhibits spin triplet resonances (|D| = 0.0118 cm–1 and E/D = 0). The extra delocalization of spin density on the azo group contributes to a lowering of the D value relative to those of Schiff base derivatives. The temperature dependence of the EPR signal and DFT calculations reveal an excited state for the spin triplet and a weak exchange coupling constant J = –2.73 cm–1

    Altered distribution and function of splenic innate lymphoid cells in adult chronic immune thrombocytopenia

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    IF 7.607International audienceInnate lymphoid cells (ILCs) have been characterized as innate immune cells capable to modulate the immune response in the mucosae. Human ILCs have been rarely described in secondary lymphoid organs except in tonsils. Moreover, their function and phenotype in human secondary lymphoid organs during autoimmune diseases have never been studied. We took advantage of splenectomy as a treatment of immune thrombocytopenia (ITP) to describe and compare splenic ILC from 18 ITP patients to 11 controls. We first confirmed that ILC3 represented the most abundant ILC subset in human non-inflamed spleens, accounting for 90% of total ILC, and that they were mostly constituted of NKp44- cells. On the contrary, proportions of ILC1 and ILC2 in spleens were lower than in blood. Splenic IL-2- and IFN-Îł-producing ILC1 were increased in ITP. While the frequencies of total splenic ILC3 were similar in the two groups, splenic GM-CSF-producing ILC3 were increased in ITP. This is the first description of human ILC in a major secondary lymphoid organ during an autoimmune disease, ITP. We observed an expansion of splenic ILC1 that could participate to the Th1 skewing, while the increased production of GM-CSF by splenic ILC3 could stimulate splenic macrophages which play a key role in ITP pathophysiology

    Supplemental Material - Risk of livedo with antiphospholipid antibodies in patients with systemic lupus erythematosus: A systematic review and meta-analysis

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    Supplemental Material for Risk of livedo with antiphospholipid antibodies in patients with systemic lupus erythematosus: A systematic review and meta-analysis by Pierre Loiseau, Thomas Foret, Ersilia M DeFilippis, Jessie Risse, Anais D Etienne, Virginie Dufrost, Thomas Moulinet, Doruk Erkan, Hervé Devilliers, Denis Wahl, and Stéphane Zuily in Lupus</p
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