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 Type
INTERVENTIONAL
Allocation
NON_RANDOMIZED
Purpose
BASIC_SCIENCE
Masking
NONE
Enrollment
120
Assistance Publique Hopitaux de Marseille
Marseille, France
the cytotoxicité lymphocytaire
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.
Time frame: 3 years
Study of the mechanisms of the activation macrophagique
Study of the mechanisms of the activation macrophagique
Time frame: 3 years
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