0000000000267044
AUTHOR
Sabine Berthier
Should mild hypogammaglobulinemia be managed as severe hypogammaglobulinemia? A study of 389 patients with secondary hypogammaglobulinemia.
Although secondary hypogammaglobulinemia is more frequent than primary hypogammaglobulinemia, its etiology and management are poorly described, particularly for mild hypogammaglobulinemia.This retrospective observational study included all adult patients with a gammaglobulin level6.4g/L on serum electrophoresis identified at Dijon teaching hospital between April and September 2012. Clinico-biological features, etiologies and infectious complications were collected at inclusion and compared between group 1 (gammaglobulin5g/L, severe hypogammaglobulinemia), and group 2 (gammaglobulin6.4 and ≥5g/L, mild hypogammaglobulinemia).Among the 4011 serum electrophoreses, 570 samples from 389 patients …
Modification de la réponse immunitaire au cours de la maladie de Rendu-Osler
Introduction La maladie de Rendu-Osler (MRO) est une maladie genetique vasculaire rare caracterisee par une neo-angiogenese deregulee aboutissant a des epistaxis anemiantes parfois associees a malformations arterio-veineuses (MAV) pulmonaires, hepatiques ou cerebrales. La MRO est egalement associee a une lymphopenie T predominant sur les CD4+ dont les causes et les consequences sont mal connues. L’objectif de ce travail est de decrire les polarisations lymphocytaires Th1, Th2, Th17, Th1-17 et Treg au cours de la MRO. Patients et methodes Les patients atteints de MRO confirmee genetiquement ont ete prospectivement inclus lors de leur suivi habituel. Le score de severite des epistaxis (ESS), …
Haemolytic-uraemic syndrome during severe lupus nephritis: efficacy of plasma exchange
Systemic lupus erythematosus (SLE) has been described as a cause of thrombotic microangiopathy, especially thrombotic thrombocytopenic purpura (TTP). Haemolytic-uraemic syndrome (HUS) is less frequent in SLE. We report a case of such an association during an episode of severe lupus nephritis in a young woman, who was successfully treated with steroids, cyclophosphamide and especially plasma exchange with plasma replacement. This report highlights the importance of recognising atypical HUS in SLE patients by looking for schistocytes in case of haemolytic anemia with a negative antiglobulin test, in order to begin plasma exchange.
Efficacy of Azacitidine (AZA) in Autoimmune and Inflammatory Disorders (AID) Associated with Myelodysplastic Syndromes (MDS) and Chronic Myelomonocytic Leukemia (CMML)
Abstract Background: AID are seen in 10-30% of MDS and CMML. After initial response to steroids, AID are often poorly controlled and steroid-sparing drugs are difficult to use due to the underlying MDS/CMML. Some case reports suggest a beneficial role of AZA treatment in AID associated to MDS/CMML. Methods: We retrospectively analyzed 22 MDS/CMML patients (pts) with AID who received AZA in French centers between January 2007 and May 2014. Results: Median age of the 22 pts was 70y (range 41-84), including 6F/16M. Diagnosis of MDS/CMML preceded AID (n=8) by a median of 17 months (mo), was concomitant with (n=7) or followed AID (n=7) by a median of 20 mo. 14 pts had lower risk IPSS and 8 highe…
Autoimmune Myopathy Due To Statin Treatment In An Elderly Woman
International audience
Caractérisation de l’anémie hémolytique auto-immune associée aux hémopathies lymphoïdes à partir du registre des hémopathies malignes de Côte d’Or
Introduction L’anemie hemolytique auto-immune (AHAI) est une cytopenie auto-immune (CAI) caracterisee par une destruction des globules rouges par des auto-anticorps diriges contre certains de leurs antigenes de surface. Son incidence est estimee entre 1 et 3/100 000 personnes-annees (PA). Les AHAI a auto-anticorps chauds sont les plus frequentes et les formes secondaires, observees dans la moitie des cas, ont tendance a augmenter avec l’âge, notamment en association aux hemopathies malignes. Les donnees de la litterature reposent cependant sur de faibles cohortes ou des centres tertiaires, avec un possible biais de recrutement. L’objectif de notre etude est de decrire la frequence de l’AHAI…
Th1 and Th17 lymphocytes expressing CD161 are implicated in giant cell arteritis and polymyalgia rheumatica pathogenesis.
International audience; OBJECTIVE: Giant cell arteritis (GCA) is the most frequently occurring vasculitis in elderly individuals, and its pathogenesis is not fully understood. The objective of this study was to decipher the role of the major CD4+ T cell subsets in GCA and its rheumatologic form, polymyalgia rheumatica (PMR). METHODS: A prospective study of the phenotype and the function of major CD4+ T cell subsets (Th1, Th17, and Treg cells) was performed in 34 untreated patients with GCA or PMR, in comparison with 31 healthy control subjects and with the 27 treated patients who remained after the 7 others withdrew. RESULTS: Compared with control subjects, patients with GCA and patients wi…
Immunologic effects of rituximab on the human spleen in immune thrombocytopenia
Abstract Immune thrombocytopenia (ITP) is an autoimmune disease with a complex pathogenesis. As in many B cell–related autoimmune diseases, rituximab (RTX) has been shown to increase platelet counts in some ITP patients. From an immunologic standpoint, the mode of action of RTX and the reasons underlying its limited efficacy have yet to be elucidated. Because splenectomy is a cornerstone treatment of ITP, the immune effect of RTX on this major secondary lymphoid organ was investigated in 18 spleens removed from ITP patients who were treated or not with RTX. Spleens from ITP individuals had follicular hyperplasia consistent with secondary follicles. RTX therapy resulted in complete B-cell de…
Caractérisation de la thrombopénie immunologique associée aux hémopathies lymphoïdes à partir du registre des hémopathies malignes de Côte d’Or
Introduction Le purpura thrombopenique immunologique (PTI) est une cytopenie auto-immune (CAI) caracterisee par une diminution de la duree de vie des plaquettes liee a la fois a leur destruction peripherique par les macrophages spleniques et a un defaut de production medullaire. Son incidence est estimee entre 3 et 4/100 000 personnes-annees (PA). S’il est le plus souvent primaire, il est associe dans 15–20 % des cas a d’autres maladies auto-immunes ou a des hemopathies malignes. Pour ces dernieres, les associations les plus frequentes sont rapportees avec la leucemie lymphoide chronique (LLC), la leucemie a grands lymphocytes T granuleux (LGL) et le lymphome de Hodgkin (LH) mais les donnee…
Efficacy and safety of dapsone as second line therapy for adult immune thrombocytopenia: A retrospective study of 42 patients.
Dapsone is recommended as a second line therapy in immune thrombocytopenia (ITP), but is underused because of its potential side effects. The medical charts of 42 ITP patients treated with dapsone (100 mg/day) were retrospectively reviewed in order to assess its efficacy and safety in daily clinical practice. The overall response rate was 54.8% (n = 22, with a complete response in 38.1%) with a median time to response of 29 days (24-41 days). Patients with complete response had shorter disease duration whereas no difference was observed between responders and non-responders regarding age, sex or previous treatments received. Importantly, after dapsone withdrawal, a sustained response was ob…
Factors Associated with Specific Quality of Life Evolution in SLE Patients: A French Prospective Longitudinal Multicenter Study
IF 7.879; International audience
Preferential splenic CD8+ T-cell activation in rituximab-nonresponder patients with immune thrombocytopenia
The pathogenic role of B cells in immune thrombocytopenia (ITP) has justified the therapeutic use of anti-CD20 antibodies such as rituximab (RTX). However, 60% of ITP patients do not respond to RTX. To decipher the mechanisms implicated in the failure of RTX, and because the spleen plays a well-recognized role in ITP pathogenesis, 12 spleens from ITP patients who had been nonresponders to RTX therapy were compared with 11 spleens from RTX-untreated ITP patients and 9 controls. We here demonstrate that in RTX-nonresponder ITP patients, preferential Th1 and Tc1 T lymphocyte polarizations occur, associated with an increase in splenic effector memory CD8(+) T-cell frequency. Moreover, in the RT…