Secondary Adult's Hemophagocytic Lymphohistiocytosis and Innate Immunity

The recruitment status of this study is unknown because the information has not been verified recently.
Verified May 2010 by Assistance Publique Hopitaux De Marseille.
Recruitment status was  Recruiting
Sponsor:
Information provided by:
Assistance Publique Hopitaux De Marseille
ClinicalTrials.gov Identifier:
NCT01125319
First received: May 17, 2010
Last updated: May 18, 2010
Last verified: May 2010
  Purpose

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 Intervention
Hemophagocytic Lymphohisticytosis
Other: blood sample

Study Type: Interventional
Study Design: Allocation: Non-Randomized
Intervention Model: Parallel Assignment
Masking: Open Label
Primary Purpose: Basic Science
Official Title: Secondary Adult's Hemophagocytic Lymphohistiocytosis and Innate Immunity

Resource links provided by NLM:


Further study details as provided by Assistance Publique Hopitaux De Marseille:

Primary Outcome Measures:
  • the cytotoxicité lymphocytaire [ Time Frame: 3 years ] [ Designated as safety issue: No ]
    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 [ Time Frame: 3 years ] [ Designated as safety issue: No ]
    Study of the mechanisms of the activation macrophagique


Estimated Enrollment: 120
Study Start Date: March 2010
Estimated Study Completion Date: September 2013
Estimated Primary Completion Date: March 2013 (Final data collection date for primary outcome measure)
Arms Assigned Interventions
patients Hemophagocytic lymphohisticytosis group Other: blood sample
group control patient Other: blood sample
healthy control group Other: blood sample

  Eligibility

Ages Eligible for Study:   18 Years and older
Genders Eligible for Study:   Both
Accepts Healthy Volunteers:   Yes
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)

  Contacts and Locations
Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the Contacts provided below. For general information, see Learn About Clinical Studies.

Please refer to this study by its ClinicalTrials.gov identifier: NCT01125319

Contacts
Contact: jean robert harle 04 91 38 33 27 jean-robert.harle@ap-hm.fr

Locations
France
Assistance Publique Hopitaux de Marseille Recruiting
Marseille, France
Contact: jean robert HARLE       jean-robert.harle@ap-hm.fr   
Principal Investigator: jean robert harle         
Sponsors and Collaborators
Assistance Publique Hopitaux De Marseille
Investigators
Principal Investigator: jean robert HARLE Assistance Publique Hopitaux De Marseille
  More Information

No publications provided

Responsible Party: Assistance Publique - Hopitaux de Marseille, Direction de la Recherche et de l'Innovation
ClinicalTrials.gov Identifier: NCT01125319     History of Changes
Other Study ID Numbers: 2007/35, 2007-A01109-44
Study First Received: May 17, 2010
Last Updated: May 18, 2010
Health Authority: France: Afssaps - Agence française de sécurité sanitaire des produits de santé (Saint-Denis)

Additional relevant MeSH terms:
Lymphohistiocytosis, Hemophagocytic
Histiocytosis, Non-Langerhans-Cell
Histiocytosis
Lymphatic Diseases

ClinicalTrials.gov processed this record on July 26, 2014