Secondary Adult's Hemophagocytic Lymphohistiocytosis and Innate Immunity
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|ClinicalTrials.gov Identifier: NCT01125319|
Recruitment Status : Completed
First Posted : May 18, 2010
Last Update Posted : July 1, 2015
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.
|Condition or disease||Intervention/treatment|
|Hemophagocytic Lymphohisticytosis||Other: blood sample|
|Study Type :||Interventional (Clinical Trial)|
|Actual Enrollment :||120 participants|
|Intervention Model:||Parallel Assignment|
|Masking:||None (Open Label)|
|Primary Purpose:||Basic Science|
|Official Title:||Secondary Adult's Hemophagocytic Lymphohistiocytosis and Innate Immunity|
|Study Start Date :||March 2010|
|Primary Completion Date :||March 2014|
|Study Completion Date :||October 2014|
|patients Hemophagocytic lymphohisticytosis group||Other: blood sample|
|group control patient||Other: blood sample|
|healthy control group||Other: blood sample|
- 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
To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.
Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT01125319
|Assistance Publique Hopitaux de Marseille|
|Principal Investigator:||jean robert HARLE||Assistance Publique Hopitaux De Marseille|