Apport du traitement substitutif par les immunoglobulines au cours des agammaglobulinémies : à propos de 10 cas

International audienceIntravenous immunoglobulin (Ig IV) has been used for many years in the treatment of primary antibody deficiencies. We performed a retrospective study of the clinical features and outcome of agammaglobulinemia children who received prolonged Ig IV infusions. Patients and methods. - Ten children, 9 male et 1 female, with agammaglobulinemia diagnosis were studied for the clinical manifestations before and during the Ig IV replacement therapy. Serum Ig levels were quantified by nephelometry. Circulating B ant T cells were counted by immuno fluorescence labeling by monoclonal antibodies. T-cell fonctions were assessed by using mitogen and antigen -induced T-cell proliferation assays in vitro. Patients clinical status was evaluated respectively, before initiation and at every moment (when patients had an infection) of the replacement therapy. Results. - Ig IV therapy was performed for 866 cumulated months, median 108 months. The median Ig IV doses administered to the 10 patients was 500 mg/kg/month. Residual serum IgG mean level was 3,9 g/L. All patients had 99 bacterial infections/ year before Ig IV, mainly respiratory tract infections (48,5%), and 4 patients had bronchiectasis before Ig replacement therapy. The number of infection /year fall to 25 during IgIV replacement, and the infection/patient/year rate decreases significantly. One patient developed an Echovirus 27 meningoencephalitis during this treatment. Conclusion. - Ig IV therapy with residual IgG mean level of 3,9 g/l reduced significantly the rate of bacterial infections. The use of specific antibiotherapy and respiratory kinesitherapy led to a lower rate of respiratory tract infections, and the stabilisation of the bronchiectasis. However this intravenous replacement therapy does not protect against viral meningoencephalitis. (c) 2006 Elsevier SAS. Tous droits reserves.L'administration par voie intraveineuse d'immunoglobulines polyvalentes (Ig IV) est utilisée, depuis de nombreuses années, dans le traitement substitutif des déficits immunitaires primitifs avec défaut de production d'anticorps. Dans cette étude, nous avons évalué le devenir d'enfants atteints par ces maladies ayant reçu ce type de traitement de façon prolongée

Renal impairment in multiple myeloma: A single center experience

To determine the features of the different forms of kidney diseases associated with multiple myeloma (MM), we retrospectively studied 144 patients with kidney injury and MM at our institute from 1974 to 2014. The mean age of the patients was 60.1 years and the male:female ratio was 1.25. Renal disease was concomitant with the discovery of MM in 92% of cases. The mean follow-up of our patients was 2.1 years. Initial renal insufficiency was found in 131 (91%) patients. Cast nephropathy of the distal tubule was found in 110 (26%) patients, renal amyloidosis in 16 (11.1%), and light chain deposition disease in five (3.47%). Twelve (8.3%) patients had chronic glomerular nephropathy. Twenty-six patients reached end-stage renal failure within 13.4 months. Renal survival was 30.45 months. Predictive factors for improvement of renal function undergoing chemotherapy included serum creatinine <250 µmol/L, proteinuria <1 g/24 h, and the non-use of renal replacement therapy. Multiple pathogenic mechanisms can contribute to kidney injury in myeloma patients. Novel myeloma agents have shown considerable promise in reversing renal failure in some patients and improving outcomes