Secondary Adult's Hemophagocytic Lymphohistiocytosis and Innate Immunity
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.
Study Design
Allocation: Non-Randomized, Intervention Model: Parallel Assignment, Masking: Open Label, Primary Purpose: Basic Science
Conditions
Hemophagocytic Lymphohisticytosis
Intervention
blood sample
Location
Assistance Publique Hopitaux de Marseille
Marseille
France
Status
Recruiting
Source
Assistance Publique Hopitaux De Marseille
Results (where available)
Links
- Source: http://clinicaltrials.gov/show/NCT01125319
- Information obtained from ClinicalTrials.gov on July 15, 2010
Medical and Biotech [MESH] Definitions
Blood Buffy Coat
The fraction of a blood sample, following CENTRIFUGATION, that is distinguished as a thin light-colored layer between the RED BLOOD CELLS, underneath it, and the PLASMA, above it. It is composed mostly of WHITE BLOOD CELLS and PLATELETS.
Erythrocyte Count
The number of RED BLOOD CELLS per unit volume in a sample of venous BLOOD.
Blood Cell Count
The number of LEUKOCYTES and ERYTHROCYTES per unit volume in a sample of venous BLOOD. A complete blood count (CBC) also includes measurement of the HEMOGLOBIN; HEMATOCRIT; and ERYTHROCYTE INDICES.
Blood Specimen Collection
The taking of a blood sample to determine its character as a whole, to identify levels of its component cells, chemicals, gases, or other constituents, to perform pathological examination, etc.
Flow Injection Analysis
The analysis of a chemical substance by inserting a sample into a carrier stream of reagent using a sample injection valve that propels the sample downstream where mixing occurs in a coiled tube, then passes into a flow-through detector and a recorder or other data handling device.
Clinical Trials
Treatment Protocol for Hemophagocytic Lymphohistiocytosis 2004
Without therapy HLH is often fatal, and often rapidly fatal. The treatment protocol HLH-94 has improved survival markedly as compared to the survival earlier. We now aim to improve surviva...
Hybrid Immunotherapy for Hemophagocytic LymphoHistiocytosis
Despite good progress during the last decade, hemophagocytic ymphohistiocytosis (HLH) remains difficult to treat. Two different treatment regimens have been used successfully. The first o...
OBJECTIVES: I. Determine the efficacy of unrelated donor hematopoietic stem cell transplantation in the treatment of patients with life threatening hemophagocytic disorders. II. Determi...
Pain Detection and Interception in Neonates
Hypothesis: 1. May the introduction of NIDCAP during blood sample reduced pain in neonates during blood sample 2. Compare the effect of venous and capillary blood sample m...
Sample Collection From Melanoma Patients
The goal of this laboratory research study is to collect blood and tumor samples from patients with malignant melanoma. Researchers want to store these samples in a secure and confidential...
PubMed Articles
Hemophagocytic syndrome in a 4-month-old infant with biotinidase deficiency.
Hemophagocytic syndromes such as hemophagocytic lymphohistiocytosis (HLH) are life-threatening hyperinflammatory conditions caused by inherited or acquired immune disorders. Awareness of the clinical...
Mucormycosis is a rare and often fatal fungal infection caused by fungi of the Mucorales order. It is mostly seen in people with weakened immune systems or with uncontrolled diabetes, and can cause ex...
Hemophagocytic lymphohistiocytosis (HLH) is difficult to diagnose and treat. Highly elevated ferritin is strongly associated with HLH and levels may provide a prognostic marker. A comprehensive review...
Hemophagocytic syndrome is a rare disorder mainly affecting children. Symptoms include prolonged fever, hepatosplenomegaly and cytopenias. Allogeneic stem cell transplant appears to provide the best o...
Measles and secondary hemophagocytic lymphohistiocytosis.
TO THE EDITOR: We found interesting the article by Lupo et al. about a case of fatal measles in an immunocompetent 29-year-old woman (Fatal measles without rash in immunocompetent adult, France; http:...
