Secondary Adult's Hemophagocytic Lymphohistiocytosis and Innate Immunity
Hemophagocytic lymphohisticytosis (HLH) is a rare and severe disease of genetic origin in children (familial-HLH, F-HLH) or affecting adults secondary to infections, hematologic malignancies or auto-immune diseases (secondary_HLH, S-HLH). F-HLH are due to genetic mutations affecting the genes of perforin or proteins involved in its secretion, resulting in the complete loss of lymphocyte cytotoxicity without affecting lymphocyte number. In S-HLH, the investigators have observed a severe NK cell lymphopenia and a transient loss of cytotoxicity of unknown mechanism. In this study, the investigators will dissect macrophage activation mechanisms as well as NK cytotoxicity inability in adults patients suffering of S-HLH.
Macrophage activation could result from the loss of the retro-control normally exerted by NK cells, thus the investigators will analyze reciprocal interactions of macrophages and NK cells during the acute phase and after recovery of S-HLH.
|Study Design:||Allocation: Non-Randomized
Intervention Model: Parallel Assignment
Masking: Open Label
Primary Purpose: Basic Science
|Official Title:||Secondary Adult's Hemophagocytic Lymphohistiocytosis and Innate Immunity|
- the cytotoxicité lymphocytaire [ Time Frame: 3 years ]To compare the cytotoxicité lymphocytaire at the patients affected by LHS with regard to 2 groups of subjects controls: a group of patients affected by infections except HIV, by hémopathies or by epithelial métastasés cancers, by autoimmunes / inflammatory pathologies (spread erythemic lupus and disease of Still of the adult) but having no LHS (controls-patients) and a group of healthy subjects.
- Study of the mechanisms of the activation macrophagique [ Time Frame: 3 years ]Study of the mechanisms of the activation macrophagique
|Study Start Date:||March 2010|
|Study Completion Date:||October 2014|
|Primary Completion Date:||March 2014 (Final data collection date for primary outcome measure)|
|patients Hemophagocytic lymphohisticytosis group||Other: blood sample|
|group control patient||Other: blood sample|
|healthy control group||Other: blood sample|
Please refer to this study by its ClinicalTrials.gov identifier: NCT01125319
|Assistance Publique Hopitaux de Marseille|
|Principal Investigator:||jean robert HARLE||Assistance Publique Hopitaux De Marseille|