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Secondary Adult's Hemophagocytic Lymphohistiocytosis and Innate Immunity

2014-08-27 03:13:39 | BioPortfolio

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)

View Results

Links

Published on BioPortfolio: 2014-08-27T03:13:39-0400

Clinical Trials [410 Associated Clinical Trials listed on BioPortfolio]

Prospective Cohort for Adult Hemophagocytosis

This prospective study enrolls subjects who have clinical and laboratory manifestations related with hemophagocytic lymphohistiocytosis. The purpose of the study is to evaluate clinical an...

Tocilizumab and Hemophagocytic Lymphohistiocytosis (HLH)

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Study to Evaluate Interferon Gamma (IFNγ) and Other Inflammatory Mediators in Patients With Malignancy-Associated Hemophagocytic Lymphohistiocytosis (M-HLH)

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Diagnostic Biomarkers for Adult Hemophagocytic Lymphohistiocytosis in Critically Ill Patients (HEMICU)

Hemophagocytic lymphohistiocytosis in adults (HLH) is at 68% mortality whereas 78% of all cases remain undiagnosed though therapies are available which clearly reduce mortality. The invest...

DEP-Ru Regimen as a Salvage Therapy for HLH

This study aimed to investigate the efficacy and safety of ruxolitinib together with liposomal doxorubicin, etoposide and methylprednisolone (DEP-Ru) as a salvage therapy for refractory/re...

PubMed Articles [10685 Associated PubMed Articles listed on BioPortfolio]

Hemophagocytic Lymphohystiocytosis Associated With Type Ia Glycogen Storage Disease.

Hemophagocytic lymphohystiocytosis (HLH) is characterized by fever, splenomegaly, pancytopenia, and elevated levels of triglycerides and ferritin. These signs and symptoms are common to other metaboli...

Dried Blood Spheroids for Dry-state Room Temperature Stabilization of Microliter Blood Samples.

It is well known that 2D dried blood spots on paper offers a facile sample collection, storage, and transportation of blood. However, large volume requirements, possible analyte instability and diffic...

Hemophagocytic Lymphohistiocytosis in Cutaneous T-Cell Lymphoma.

Hemophagocytic lymphohistiocytosis (HLH) has been reported as a serious complication of cutaneous T-cell lymphoma (CTCL). Despite available diagnostic guidelines, it remains a diagnostic and therapeut...

A successful treatment of severe systemic lupus erythematosus caused by occult pulmonary infection-associated with hemophagocytic syndrome: A case report.

A 27-year-old woman with a history of systemic lupus erythaematosus (SLE) developed hemophagocytic syndrome (HPS) secondary due to an unrecognized infection that led to severe SLE with a prolonged rec...

Hemophagocytic Lymphohistiocytosis Associated With Visceral Leishmaniasis.

This is the report of a 2-year-old boy who presented with fever, cytopenia, and splenomegaly. The patient was diagnosed with hemophagocytic lymphohistiocytosis (HLH) and treated with HLH-2004 protocol...

Medical and Biotech [MESH] Definitions

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.

The number of RED BLOOD CELLS per unit volume in a sample of venous BLOOD.

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.

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.

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.

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