Maladie de Rendu-Osler et abcès hépatique

Bui, E.; Gorse, Audrey; Prelipcean, V.; Lionetto, F.; Forestier, E.; Camuset, G.; Hansmann, Yves; Christmann, D.

doi:10.1016/j.medmal.2009.02.012

Cited by 1 publication

(1 citation statement)

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“…Serious infections in patients with HHT are clinically relevant events, documented in many case reports . In a retrospective study, about 13% of patients in an HHT cohort had a history of severe infection, in particular cerebral abscesses or staphylococcal osteoarthritis.…”

Section: Introductionmentioning

confidence: 99%

Immunological abnormalities associated with hereditary haemorrhagic telangiectasia

Guilhem

Malcus

Clarivet³

et al. 2013

J Intern Med

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Abstract. Guilhem A, Malcus C, Clarivet B, Plauchu H, Dupuis-Girod S (Hôpital Saint Eloi, Montpellier; Hôpital E Herriot, Lyon; Hôpital E Herriot, Lyon; Hôpital La Colombiere, Montpellier; Centre National de R ef erence pour la Maladie de Rendu-Osler, Hôpital Louis Pradel, Bron, France). Immunological abnormalities associated with hereditary haemorrhagic telangiectasia. J Intern Med 2013; 274: 351-362.Objective. Hereditary haemorrhagic telangiectasia (HHT) is a genetic disorder related to mutations in one of the coreceptors to the transforming growth factor-b superfamily (ALK1 or endoglin). Besides the obvious vascular symptoms (epistaxis and arteriovenous malformations), patients have an unexplained high risk of severe bacterial infections. The aim of the study was to assess the main immunological functions of patients with HHT using the standard biological tests for primary immunodeficiencies.Design, setting and subjects. A prospective single-centre study of 42 consecutive adult patients with an established diagnosis of HHT was conducted at the National French HHT Reference Center (Lyon). Lymphocyte subpopulations and proliferation capacity, immunoglobulin levels and neutrophil and monocyte phagocytosis, oxidative burst and chemotaxis were assessed.Results. Innate immunity was not altered in patients with HHT. With regard to adaptive immunity, significant changes were seen in immunological parameters: primarily, a lymphopenia in patients with HHT compared with healthy control subjects affecting mean CD4 (642 cells lL À1 vs. 832 cells lL À1, P < 0.001), CD8 (295 cells lL À1 vs. 501 cells lL À1 , P < 0.0001) and natural killer (NK) cells (169 cells lL À1 vs. 221 cells lL À1 , P < 0.01), associated with increased levels of immunoglobulins G and A. This lymphopenia mainly concerned na€ ıve T cells. Proliferation capacities of lymphocytes were normal. Lymphopenic patients had a higher frequency of iron supplementation but no increase in infection rate. Lower levels of immunoglobulin M and a higher rate of pulmonary arteriovenous malformations were found amongst patients with a history of severe infection.Conclusions. Patients with HHT exhibit immunological abnormalities including T CD4, T CD8 and NK cell lymphopenia and increased levels of immunoglobulins G and A. The observed low level of immunoglobulin M requires further investigation to determine whether it is a specific risk factor for infection in HHT.

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Section: Introductionmentioning

confidence: 99%