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Secondary Adult's Hemophagocytic Lymphohistiocytosis and Innate Immunity

Primary Purpose

Hemophagocytic Lymphohisticytosis

Status

Completed

Phase

Not Applicable

Locations

France

Study Type

Interventional

Intervention

blood sample

About this trial

This is an interventional basic science trial for Hemophagocytic Lymphohisticytosis

Eligibility Criteria

18 Years - undefined (Adult, Older Adult)All SexesAccepts Healthy Volunteers

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 guardianship and to express their consent)
histories of established LHF or episode of LH during the childhood
patients reached(affected) by infection by the HIV: either histories known for seropositivity and / or of disease, or because of the positivity of the test sérologique realized in the inclusion after consent of the patient.
Have already benefited from a specific treatment of the LHS

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)

Patients answering the criteria of diagnosis of LHS
Patients having already had histories of LH
Patients having already received the specific treatments of these diseases
patients reached(affected) by infection by the HIV: either histories known for seropositivity and / or of disease, or because of the positivity of the test sérologique realized in the inclusion after consent of the patient.

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)

Sites / Locations

Assistance Publique Hopitaux de Marseille

Arms of the Study

Arm 1

Arm 2

Arm 3

Arm Type

Other

Arm Label

patients Hemophagocytic lymphohisticytosis group

group control patient

healthy control group

Arm Description

Outcomes

Primary Outcome Measures

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.

Secondary Outcome Measures

Study of the mechanisms of the activation macrophagique

Full Information

NCT ID

NCT01125319

First Posted

May 17, 2010

Last Updated

June 30, 2015

Sponsor

Assistance Publique Hopitaux De Marseille

1. Study Identification

Unique Protocol Identification Number

NCT01125319

Brief Title

Secondary Adult's Hemophagocytic Lymphohistiocytosis and Innate Immunity

Official Title

Secondary Adult's Hemophagocytic Lymphohistiocytosis and Innate Immunity

Study Type

Interventional

2. Study Status

Record Verification Date

June 2015

Overall Recruitment Status

Completed

Study Start Date

March 2010 (undefined)

Primary Completion Date

March 2014 (Actual)

Study Completion Date

October 2014 (Actual)

3. Sponsor/Collaborators

Responsible Party, by Official Title

Sponsor

Name of the Sponsor

Assistance Publique Hopitaux De Marseille

4. Oversight

Data Monitoring Committee

5. Study Description

Brief 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.

6. Conditions and Keywords

Primary Disease or Condition Being Studied in the Trial, or the Focus of the Study

Hemophagocytic Lymphohisticytosis

7. Study Design

Primary Purpose

Basic Science

Study Phase

Not Applicable

Interventional Study Model

Parallel Assignment

Masking

None (Open Label)

Allocation

Non-Randomized

Enrollment

120 (Actual)

8. Arms, Groups, and Interventions

Arm Title

patients Hemophagocytic lymphohisticytosis group

Arm Type

Other

Arm Title

group control patient

Arm Type

Other

Arm Title

healthy control group

Arm Type

Other

Intervention Type

Other

Intervention Name(s)

blood sample

Primary Outcome Measure Information:

Title

the cytotoxicité lymphocytaire

Description

Time Frame

3 years

Secondary Outcome Measure Information:

Title

Study of the mechanisms of the activation macrophagique

Description

Study of the mechanisms of the activation macrophagique

Time Frame

3 years

10. Eligibility

Sex

All

Minimum Age & Unit of Time

18 Years

Accepts Healthy Volunteers

Eligibility Criteria

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 guardianship and to express their consent) histories of established LHF or episode of LH during the childhood patients reached(affected) by infection by the HIV: either histories known for seropositivity and / or of disease, or because of the positivity of the test sérologique realized in the inclusion after consent of the patient. Have already benefited from a specific treatment of the LHS 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) Patients answering the criteria of diagnosis of LHS Patients having already had histories of LH Patients having already received the specific treatments of these diseases patients reached(affected) by infection by the HIV: either histories known for seropositivity and / or of disease, or because of the positivity of the test sérologique realized in the inclusion after consent of the patient. 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)

Overall Study Officials:

First Name & Middle Initial & Last Name & Degree

jean robert HARLE

Organizational Affiliation

Assistance Publique Hopitaux De Marseille

Official's Role

Principal Investigator

Facility Information:

Facility Name

Assistance Publique Hopitaux de Marseille

City

Marseille

Country

France

12. IPD Sharing Statement

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Secondary Adult's Hemophagocytic Lymphohistiocytosis and Innate Immunity

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