Evaluation of IFNγ and Inflammatory Mediators in Patients With Hemophagocytic Lymphohistiocytosis
- Conditions
- Hemophagocytic Lymphohistiocytoses
- Interventions
- Other: Blood Draws Data Collection
- Registration Number
- NCT06047210
- Lead Sponsor
- Swedish Orphan Biovitrum
- Brief Summary
This observational study is designed to provide a better understanding of the potential use of interferon gamma (IFNγ) and IFNγ-inducible chemokines as markers of HLH disease activity in adults, and the potential of IFNγ to represent a therapeutic target.
- Detailed Description
This is a observational study designed to determine the levels of pro-inflammatory markers in adult patients diagnosed with HLH and to assess the relationship between the biomarkers and disease activity in these patients during the HLH course. Inflammatory markers include IFNγ and IFNγ-induced chemokines C-X-C chemokine ligand 9 and C-X-C chemokine ligand 10.
In addition to the blood samples for the biomarker analysis, relevant information gathered by the treating physician will be collected in a data collection form. Whenever possible, collection of serum samples for biomarker analysis and relevant information should occur at HLH diagnosis, at regular time intervals during the treatment course (not more than once a week) up to resolution of HLH.
Blood samples will also be sent to the study sponsor (NovImmune S.A.) for cytokine testing. Before patient's samples are sent to the sponsor for testing, patient's name and any personal identifying information will be coded to protect participant's privacy.
Recruitment & Eligibility
- Status
- COMPLETED
- Sex
- All
- Target Recruitment
- 14
- Patients >=18 years old diagnosed with active HLH as established by the treating physician.
- HLH forms of unknown origin or secondary to infections or rheumatologic disorders.
- Should the diagnosis of primary HLH or malignancy become apparent after inclusion, the data collected will be analyzed separately as additional cohorts.
- The patient must have consented to the use of their clinical data for research purposes at the site.
- Patients with primary HLH (diagnosed by the presence of homozygous mutations in a known HLH causative gene) and secondary HLH due to malignancy are excluded.
Study & Design
- Study Type
- OBSERVATIONAL
- Study Design
- Not specified
- Arm && Interventions
Group Intervention Description A-HLH Patients Blood Draws Data Collection Patients diagnosed with A-HLH. Malignancy-Associated Hemophagocytic Lymphohistiocytosis (M-HLH) Patients Blood Draws Data Collection Patients diagnosed with HLH in the context of a malignancy.
- Primary Outcome Measures
Name Time Method Mean Serum Concentration of Inflammatory Biomarker - C-X-C Chemokine Ligand 10 (CXCL10) 11 months Serum samples was collected at various times after HLH diagnosis to determine the concentration of CXCL10.
Mean Serum Concentration of Inflammatory Biomarker - C-X-C Chemokine Ligand 9 (CXCL9) 11 months Serum samples was collected at various times after HLH diagnosis to determine the concentration of CXCL9.
Mean Serum Concentration of Inflammatory Biomarker - Interferon Gamma (IFNg) 11 months Serum samples was collected at various times after HLH diagnosis to determine the concentration of IFNg.
Mean Serum Concentration of Inflammatory Biomarker - Interleukin 10 (IL-10) 11 months Serum samples was collected at various times after HLH diagnosis to determine the concentration of IL-10.
Mean Serum Concentration of Inflammatory Biomarker - Interleukin 1 Beta (IL-1B) 11 months Serum samples was collected at various times after HLH diagnosis to determine the concentration of IL-1B.
Mean Serum Concentration of Inflammatory Biomarker - Interleukin 6 (IL-6) 11 months Serum samples was collected at various times after HLH diagnosis to determine the concentration of IL-6.
Mean Serum Concentration of Inflammatory Biomarker - Neopterin 11 months Serum samples was collected at various times after HLH diagnosis to determine the concentration of neopterin.
Mean Serum Concentration of Inflammatory Biomarker - Tumor Necrosis Factor Alpha (TNFa) 11 months Serum samples was collected at various times after HLH diagnosis to determine the concentration of TNFa.
Mean Serum Concentration of Inflammatory Biomarker - Total Human Interferon Gamma (hIFNg) 11 months Serum samples was collected at various times after HLH diagnosis to determine the concentration of hIFNg.
Mean Serum Concentration of Inflammatory Biomarker - Soluble CD163 (sCD163) 11 months Serum samples was collected at various times after HLH diagnosis to determine the concentration of sCD163.
Mean Serum Concentration of Inflammatory Biomarker - Soluble IL-2 Receptor Alpha (sIL2Ra) 11 months Serum samples was collected at various times after HLH diagnosis to determine the concentration of sIL2Ra.
Mean Serum Concentration of White Blood Cells 11 months Blood samples was collected at various times after HLH diagnosis to determine the concentration of white blood cells.
Mean Serum Concentration of Red Blood Cells 11 months Blood samples was collected at various times after HLH diagnosis to determine the concentration of red blood cells.
Mean Serum Concentration of Hemoglobin 11 months Blood samples was collected at various times after HLH diagnosis to determine the concentration of hemoglobin.
Mean Serum Concentration of Hematocrit 11 months Blood samples was collected at various times after HLH diagnosis to determine the concentration of hematocrit.
Mean Serum Concentration of Platelets 11 months Blood samples was collected at various times after HLH diagnosis to determine the concentration of platelets.
Mean Serum Concentration of Neutrophils 11 months Blood samples was collected at various times after HLH diagnosis to determine the concentration of neutrophils.
Mean Serum Concentration of Lymphocytes 11 months Blood samples was collected at various times after HLH diagnosis to determine the concentration of lymphocytes.
Mean Serum Concentration of Monocytes 11 months Blood samples was collected at various times after HLH diagnosis to determine the concentration of monocytes.
Mean Serum Concentration of Eosinophils 11 months Blood samples was collected at various times after HLH diagnosis to determine the concentration of eosinophils.
Mean Serum Concentration of Basophils 11 months Blood samples was collected at various times after HLH diagnosis to determine the concentration of basophils.
Mean Plasma Concentration of D-Dimer 11 months Blood samples was collected at various times after HLH diagnosis to determine the concentration of D-dimer.
Mean Plasma Concentration of Fibrinogen 11 months Blood samples was collected at various times after HLH diagnosis to determine the concentration of fibrinogen.
Mean Activated Partial Thromboplastin Time (aPTT) 11 months Blood samples was collected at various times after HLH diagnosis to determine the aPTT.
Mean Prothrombin Time 11 months Blood samples was collected at various times after HLH diagnosis to determine the prothrombin time.
Mean Serum Concentration of C-Reactive Protein 11 months Blood samples was collected at various times after HLH diagnosis to determine the concentration of c-reactive protein.
Mean Serum Concentration of Ferritin 11 months Blood samples was collected at various times after HLH diagnosis to determine the concentration of ferritin.
Mean Plasma Concentration of Blood Urea Nitrogen 11 months Blood samples was collected at various times after HLH diagnosis to determine the concentration of blood urea nitrogen.
Mean Serum Concentration of Creatinine 11 months Blood samples was collected at various times after HLH diagnosis to determine the concentration of creatinine.
Mean Serum Concentration of Albumin 11 months Blood samples was collected at various times after HLH diagnosis to determine the concentration of albumin.
Mean Serum Concentration of Sodium 11 months Blood samples was collected at various times after HLH diagnosis to determine the concentration of sodium.
Mean Serum Concentration of Fasting Triglycerides 11 months Blood samples was collected at various times after HLH diagnosis to determine the concentration of fasting triglycerides.
Mean Serum Concentration of Total Bilirubin 11 months Blood samples was collected at various times after HLH diagnosis to determine the concentration of total bilirubin.
Mean Serum Concentration of Conjugated Bilirubin 11 months Blood samples was collected at various times after HLH diagnosis to determine the concentration of conjugated bilirubin.
Mean Serum Concentration of Alanine Aminotransferase 11 months Blood samples was collected at various times after HLH diagnosis to determine the concentration of alanine aminotransferase.
Mean Serum Concentration of Aspartate Aminotransferase 11 months Blood samples was collected at various times after HLH diagnosis to determine the concentration of aspartate aminotransferase.
Mean Serum Concentration of Alkaline Phosphatase 11 months Blood samples was collected at various times after HLH diagnosis to determine the concentration of alkaline phosphatase.
Mean Serum Concentration of Lactate Dehydrogenase 11 months Blood samples was collected at various times after HLH diagnosis to determine the concentration of lactate dehydrogenase.
Mean Serum Concentration of Immunoglobulin G (IgG) 11 months Blood samples was collected at various times after HLH diagnosis to determine the concentration of IgG.
- Secondary Outcome Measures
Name Time Method
Trial Locations
- Locations (1)
Brigham and Women's Hospital
🇺🇸Boston, Massachusetts, United States