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| ID | Type | Description | Link |
|---|---|---|---|
| 2007-A01109-44 | Registry Identifier | 2007/35 |
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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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| patients Hemophagocytic lymphohisticytosis group | Other |
| |
| group control patient | Other |
| |
| healthy control group | Other |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| blood sample | Other |
|
| Measure | Description | Time Frame |
|---|---|---|
| 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. | 3 years |
| Measure | Description | Time Frame |
|---|---|---|
| Study of the mechanisms of the activation macrophagique | Study of the mechanisms of the activation macrophagique | 3 years |
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Inclusion Criteria:
Patients Hemophagocytic lymphohisticytosis group:
group control
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:
group control
Patients under age 18 and not to belong in one group of vulnerable persons (encircled women, private persons of freedom judicially or administratively, adults under guardianship, under guardianship and to express their consent)
group healthy control Patients under age 18 and not to belong in one group of vulnerable persons (encircled women, private persons of freedom judicially or administratively, adults under guardianship, under guardianship and to express their consent)
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| Name | Affiliation | Role |
|---|---|---|
| jean robert HARLE | Assistance Publique Hopitaux De Marseille | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Assistance Publique Hopitaux de Marseille | Marseille | France |
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| ID | Term |
|---|---|
| D001800 | Blood Specimen Collection |
| ID | Term |
|---|---|
| D013048 | Specimen Handling |
| D019411 | Clinical Laboratory Techniques |
| D019937 | Diagnostic Techniques and Procedures |
| D003933 | Diagnosis |
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| D011677 | Punctures |
| D013514 | Surgical Procedures, Operative |
| D008919 | Investigative Techniques |