An observational study in Hemophagocytic Syndrome, sponsored by Assistance Publique - Hôpitaux de Paris. Completed at 1 site in France. Open to participants aged 2 Years and older. Per ClinicalTrials.gov, last updated 2025-11-20.
Sponsored by Assistance Publique - Hôpitaux de Paris · Observational
Different study of HLHa patients :
Formation of a prospective and retrospective infant, adolescent and adult HLH patients cohort.
Collection of clinical and biological, therapeutics, informations, in a register, The collection of information is:
Background:
The hemophagocytic syndrome in infant, adolescent and adults (HLH) is a serious and often lethal condition. The study of literature series HLHa shows that these syndromes frequently develop in immunocompromised patients (renal transplant, HIV, collagen in Processing immunosuppressants) in the course of a viral infection. HLH syndrome has also been described as a clinical form of lymphoma or connective disease (lupus). These clinical forms are rare, severe and recurrent suggesting the possibility that immune deficiency could be involved. The study of pediatric forms has definitely established a link between HLH syndrome and the presence of immune deficiency by identifying the nature of the latter. Four genetically determined diseases are manifested by HLH syndrome. These conditions are Family lymphohistiocytosis (LHF) syndrome, Chediak-Higashi CHS syndrome, Griscelli (GS) type 2 syndromes and X-linked lymphoproliferative (XLP 1 and 2). The mutated genes are respectively perforin Unc 13.4 and syntaxin in the LHF2, 3, 4 (10q locus genetic for LHF 1), CHS1/LYST (Lysosomal Trafficking regulator) in the CHS, in the Rab27a GS type 2, and XIAP and SH2D1A in the XLP. It is now well established that proteins encoded by these genes are necessary for the cytotoxic function of CD8 + and in the absence of these proteins is the cytotoxocity CD8 + deficient. Also, closed clinical and biological characteristics shared by pediatric genetic and adult forms suggest the existence of immune defects responsible for some or all HLH adult patients.
99 studies on the registry are indexed under Lymphohistiocytosis, Hemophagocytic; 36 are open to participants now.
This study's enrollment of 204 is above the median of 128 across 29 observational studies indexed under Lymphohistiocytosis, Hemophagocytic.
Browse Lymphohistiocytosis, Hemophagocytic studies →Assistance Publique - Hôpitaux de Paris is the lead sponsor of 3,505 studies on the registry; 1,006 are open to participants now.
Counted across the registry records on this site, refreshed daily.
Infant, Adolescent and adult patients who have a HLH syndrome regardless of etiology, hospitalized in Internal Medicine, Critical Care, Hematology, Rheumatology Neurology or Organ Transplantation
Major criteria:
Minor criteria:
These criteria will be those used for the diagnosis of HLH in adults:
One major criterion and two minor (including hyper ferritin or hypertriglyceridemia) 3 minor criteria (including hyper ferritin or hypertriglyceridemia)
Exclusion Criteria:
patient with hemophagocytic syndrome
Biological: Identification of biological markers
biologicals criteria
measure of : cytokines expression (mmol/L) Hemoglobin (g/dl) number of Platelets (number/L) number of Neutrophils (number/L) number of triglycerides (mmol/L) number of fibrinogen (g/L) number of Ferritin (microg/L)
Time frame: T0 (before traitment
name of treatment
administrated treatments
Time frame: T2 (T2 is the first day of treatment)
Clinicals criteria
clinicals description of patients : Fever, Splenomegaly and adenopathy
Time frame: T0
biologicals criteria
measure of : cytokines expression Hemoglobin level number of Platelets number of Neutrophils number of triglycerides\> number of fibrinogen number of Ferritin
Time frame: T1 (T1 is the first day of HLH syndrome)
biologicals criteria
measure of : cytokines expression Hemoglobin level number of Platelets number of Neutrophils number of triglycerides\> number of fibrinogen number of Ferritin
Time frame: T2 (T2 is the first day of treatment)
biologicals criteria
measure of : cytokines expression Hemoglobin level number of Platelets number of Neutrophils number of triglycerides\> number of fibrinogen number of Ferritin
Time frame: T4 (6 /12 months after the resolution of HLH)
Clinicals criteria
clinicals description of patients : Fever, Splenomegaly and adenopathy
Time frame: T1(T1 is the first day of HLH syndrome)
Clinicals criteria
clinicals description of patients : Fever, Splenomegaly and adenopathy
Time frame: T2 (T2 is the first day of treatment)
Clinicals criteria
clinicals description of patients : Fever, Splenomegaly and adenopathy
Time frame: T4 6 /12 months after the resolution of HLH)
name of treatment
administrated treatments
Time frame: T4(6/12 month after resolution of HLH)
This study is completed, as verified in Sep 2025. You cannot join it, but the record below documents what was studied.
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Lymphohistiocytosis, Hemophagocytic→
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