C. Gourguechon
A5076846925- Mast cells and histamine
- Immunodeficiency and Autoimmune Disorders
- Blood groups and transfusion
- Chronic Lymphocytic Leukemia Research
- Eosinophilic Disorders and Syndromes
- Multiple Myeloma Research and Treatments
- Urticaria and Related Conditions
- Inflammatory Myopathies and Dermatomyositis
- Metabolism and Genetic Disorders
- Cell Adhesion Molecules Research
- Cutaneous lymphoproliferative disorders research
- Blood disorders and treatments
- Platelet Disorders and Treatments
- Ocular Diseases and Behçet’s Syndrome
- Blood Coagulation and Thrombosis Mechanisms
- Renal Diseases and Glomerulopathies
- Glycogen Storage Diseases and Myoclonus
- Immune Cell Function and Interaction
- Alcoholism and Thiamine Deficiency
- Viral-associated cancers and disorders
- Erythrocyte Function and Pathophysiology
- Eosinophilic Esophagitis
- Amyotrophic Lateral Sclerosis Research
- Myeloproliferative Neoplasms: Diagnosis and Treatment
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
Centre Hospitalier Universitaire Amiens-Picardie
2014-2024
Centre Hospitalier d'Albi
2019-2021
Hôpital Nord
2012-2018
Université de Picardie Jules Verne
2015-2017
Centre Hospitalier Compiègne-Noyon
2012
BackgroundMastocytosis and monoclonal mast cell (MC) activation syndrome (MMAS) are heterogeneous conditions characterized by the accumulation of atypical MCs. Despite recurrent involvement KIT mutations, pathophysiologic origin mastocytosis MMAS is unclear. Although hereditary α-tryptasemia (HαT, related to TPSAB1 gene duplication) abnormally frequent in these diseases, it not known whether association coincidental or causal.ObjectiveWe evaluated prevalence HαT all subtypes assessed with...
We investigated using a custom NGS panel of 149 genes the mutational landscape 64 consecutive adult patients with tyrosine kinase fusion-negative hypereosinophilia (HE)/hypereosinophilic syndrome (HES) harboring features suggestive myeloid neoplasm. At least one mutation was reported in 50/64 (78%) (compared to 8/44 (18%) idiopathic HE/HES/HE
The aim was to describe the regulatory B and T cells (Breg Treg) helper 17 (Th17) lymphocytes before under treatment with biologic drugs, assess their potential predictive value as biomarkers of response in rheumatoid arthritis (RA). This a non-randomised, single-centre, prospective study. Patients active RA (American College Rheumatology (ACR)/European League Against Rheumatism (EULAR) 2010) who required initiation or switch any drug except rituximab were included. main judgement criterion...
We report the 11 cases of +8-MDS/MPN associated with Behcet's-like syndrome and compare them Behcet's disease Crohn's disease, pool literature for analysis. Data patients Behçet's-like were collected from MINHEMON. Eleven had (median age 75 years [IQR 65–87]; M/F ratio 0.8). MDS diagnosed at same time (7/11, 64%). By comparison 63 idiopathic without MDS, those older vs 48 years; p = .0003) less pseudofolliculitis (11% 62%; .0045) ocular impairment (0% 52%; .0008), but more frequent...
Acquired haemophilia A (AHA) is a rare haemorrhagic disease characterised by new-onset symptoms associated with dramatic decrease in factor VIII levels and an anti-factor neutralising autoantibody concentration >0.6 Bethesda units. Elderly people are often affected, whereas children rarely affected; the paediatric incidence reported literature about 0.045 case/million/year. For some time, standard of care has been that for adults, but clinicians have poor outcomes. Here, we describe largest...
We report cases of myelodysplastic syndrome/myeloproliferative neoplasms (MDS/MPN) with trisomy 8 associated inflammatory and autoimmune diseases (IADs).Data for 21 patients 8-MDS/MPN IADs were analyzed compared to 103 without IADs.The median age MDS/MPN was 67 [59-80]. The Behçet's-like disease in 11 (52%) patients, arthritis 4 (19%) Sjögren's syndrome, hemolytic anemia, aseptic abscess, periarteritis nodosa, Sweet's syndrome unclassified vasculitis one patient each. Overall, 17/21 (81%)...
Systemic mastocytosis (SM) corresponds to a rare and heterogeneous spectrum of diseases characterized by the accumulation atypical mast cells (MCs). Advanced (Adv-SM) is associated with poor survival; in contrast, patients non-advanced SM (non-Adv-SM) usually have normal life expectancy but may experience quality life. Despite recent therapeutic progress including tyrosine kinase inhibitors, new treatment options are needed for refractory and/or intolerant both severely symptomatic Adv-SM....
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A 70-year-old patient was addressed in October 2010 as part of an etiological work-up for chronic sinusitis, hyperproteinemia at 147 g/l (normal 57–82 g/l) with a peak 68.8 on serum IgG lambda protein electrophoresis. Multiple myeloma diagnosed bone marrow aspiration translocation t(4;14) cytogenetics analysis, which confers intermediate prognosis if no additional cytogenetic abnormalities. From to 2022, the received multiple lines treatment including proteasome inhibitors (bortezomib,...
Multiple acyl-coA dehydrogenase deficiency (MADD) is a rare, inherited, autosomal-recessive disorder leading to the accumulation of acylcarnitine all chain lengths. Acute decompensation with cardiac, respiratory or hepatic failure and metabolic abnormalities may be life-threatening.A 29-year-old woman presented severe lactic acidosis associated intense myalgia muscle weakness. The clinical examination revealed symmetric upper lower limb motor impairment (rated at 2 3 out 5 on Medical...
Abstract Background and Aims Systemic mastocytosis (SM) is characterized by the accumulation of atypical mast cells (MCs) in organs. Liver histology SM has been marginally described accurate histological classification critical, given consequences aggressive diagnosis. We aimed to describe features associated with liver using updated tools. Methods Using database French Reference Centre for Mastocytosis, we retrospectively identified patients a biopsy (LB) diagnosis SM. All LB procedures...
Abstract Advanced systemic mastocytosis (AdvSM) encompasses heterogeneous subtypes and is associated with poor outcomes. Although midostaurin was the first tyrosine kinase inhibitor to be approved for AdvSM patients, long‐lasting responses are limited. The mutation‐Adjusted Risk Score (MARS), International Prognostic Scoring System (IPSM) Global Systemic Mastocytosis (GPSM) have been established characterize outcomes of patients overall AdvSM. However, given outcome's dependency on subtype,...
Les cytopénies auto-immunes sont caractérisées par une destruction immunologique des éléments figurés du sang. Le réanimateur peut être confronté à ces pathologies, soit en raison de la sévérité cytopénie ou l’affection sous-jacente ; mais également l’évoquer devant cytopénie, qui est anomalie biologique fréquente réanimation et dont les étiologies peuvent multiples intriquées, rendant le diagnostic auto-immune parfois complexe. Elles partagent, outre leur rareté, l’association nombreuses...
We report the case of an 80-year-old woman who presented one episode cardiopulmonary arrest and two episodes acute airway obstruction. found in this patient presence tracheomalacia caused by megaesophagus compression secondary to achalasia probably responsible for obstruction arrest.