CompletedNot Applicable

Secondary Adult's Hemophagocytic Lymphohistiocytosis and Innate Immunity

France120 participantsStarted 2010-03

Plain-language summary

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.

Who can participate

Age range18 Years

SexALL

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AI-rewrites the medical criteria so a patient or caregiver can understand them. Always confirm with the trial site.

Inclusion Criteria: Patients Hemophagocytic lymphohisticytosis group: * Of more than 18 years old * affected by secondary LH infections with the exception of the HIV, the lymphomes, the néoplasies or the autoimmunes / inflammatory diseases following the criteria of the HLH Society modified in 2004 group control * Of more than 18 years old * At the time of the diagnosis either of firstly viral infection (EBV, CMV, parvovirus B19, HSV), is of lymphome cunning(malignant) not - Hodgkinien or of epithelial métastasés cancers, or auto-immune disease (erythemic lupus and disease of Still of the adult only) without associated criteria corresponding to the diagnosis of LHS * have agreed to benefit from a pregnancy test and from a screening sérologique by the infection by the HIV. These tests that must be negative. In case of positivity of the screening HIV, the patients will be directed to an adequate service. * Have still received no preliminary treatment for this pathology * Have agreed to participate in the study and to have signed the form of consent group healthy control They will have to be unhurt of active infectious pathology, hématologique, auto-immune or neoplastic and have by receipt of immunosuppresseurs treatments during the last 2 years. Exclusion Criteria: Patients Hemophagocytic lymphohisticytosis group: * Minor patients under age 18 or vulnerable (encircled women, private persons of freedom judicially or administratively, adults under guardianship, under guard…

What they're measuring

the cytotoxicité lymphocytaire

Timeframe: 3 years

Trial details

NCT IDNCT01125319

SponsorAssistance Publique Hopitaux De Marseille

Sponsor typeOTHER

Study typeINTERVENTIONAL

Primary completion2014-03