Evaluation of IFNγ and Inflammatory Mediators in Patients With Hemophagocytic Lymphohistiocytosis

Swedish Orphan Biovitrum14 enrolled

Overview

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.

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.

Study Type

OBSERVATIONAL

Enrollment

Conditions

Hemophagocytic Lymphohistiocytoses

Interventions

Blood Draws Data CollectionOTHER

Eligibility

Sex: ALLMin age: 18 Years

Medical Language ↔ Plain English

Inclusion Criteria: * 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. Exclusion Criteria: * 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.

Locations (1)

Brigham and Women's Hospital

Boston, Massachusetts, United States

Outcomes

Primary Outcomes

Mean Serum Concentration of Inflammatory Biomarker - C-X-C Chemokine Ligand 10 (CXCL10)

Serum samples was collected at various times after HLH diagnosis to determine the concentration of CXCL10.

Time frame: 11 months

Mean Serum Concentration of Inflammatory Biomarker - C-X-C Chemokine Ligand 9 (CXCL9)

Serum samples was collected at various times after HLH diagnosis to determine the concentration of CXCL9.

Time frame: 11 months

Mean Serum Concentration of Inflammatory Biomarker - Interferon Gamma (IFNg)

Serum samples was collected at various times after HLH diagnosis to determine the concentration of IFNg.

Time frame: 11 months

Mean Serum Concentration of Inflammatory Biomarker - Interleukin 10 (IL-10)

Serum samples was collected at various times after HLH diagnosis to determine the concentration of IL-10.

Time frame: 11 months

Mean Serum Concentration of Inflammatory Biomarker - Interleukin 1 Beta (IL-1B)

Serum samples was collected at various times after HLH diagnosis to determine the concentration of IL-1B.

Time frame: 11 months

Mean Serum Concentration of Inflammatory Biomarker - Interleukin 6 (IL-6)

Serum samples was collected at various times after HLH diagnosis to determine the concentration of IL-6.

Time frame: 11 months

Mean Serum Concentration of Inflammatory Biomarker - Neopterin

Serum samples was collected at various times after HLH diagnosis to determine the concentration of neopterin.

Time frame: 11 months

Data from ClinicalTrials.gov

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Mean Serum Concentration of Inflammatory Biomarker - Tumor Necrosis Factor Alpha (TNFa)

Serum samples was collected at various times after HLH diagnosis to determine the concentration of TNFa.

Time frame: 11 months

Mean Serum Concentration of Inflammatory Biomarker - Total Human Interferon Gamma (hIFNg)

Serum samples was collected at various times after HLH diagnosis to determine the concentration of hIFNg.

Time frame: 11 months

Mean Serum Concentration of Inflammatory Biomarker - Soluble CD163 (sCD163)

Serum samples was collected at various times after HLH diagnosis to determine the concentration of sCD163.

Time frame: 11 months

Mean Serum Concentration of Inflammatory Biomarker - Soluble IL-2 Receptor Alpha (sIL2Ra)

Serum samples was collected at various times after HLH diagnosis to determine the concentration of sIL2Ra.

Time frame: 11 months

Mean Serum Concentration of White Blood Cells

Blood samples was collected at various times after HLH diagnosis to determine the concentration of white blood cells.

Time frame: 11 months

Mean Serum Concentration of Red Blood Cells

Blood samples was collected at various times after HLH diagnosis to determine the concentration of red blood cells.

Time frame: 11 months

Mean Serum Concentration of Hemoglobin

Blood samples was collected at various times after HLH diagnosis to determine the concentration of hemoglobin.

Time frame: 11 months

Mean Serum Concentration of Hematocrit

Blood samples was collected at various times after HLH diagnosis to determine the concentration of hematocrit.

Time frame: 11 months

Mean Serum Concentration of Platelets

Blood samples was collected at various times after HLH diagnosis to determine the concentration of platelets.

Time frame: 11 months

Mean Serum Concentration of Neutrophils

Blood samples was collected at various times after HLH diagnosis to determine the concentration of neutrophils.

Time frame: 11 months

Mean Serum Concentration of Lymphocytes

Blood samples was collected at various times after HLH diagnosis to determine the concentration of lymphocytes.

Time frame: 11 months

Mean Serum Concentration of Monocytes

Blood samples was collected at various times after HLH diagnosis to determine the concentration of monocytes.

Time frame: 11 months

Mean Serum Concentration of Eosinophils

Blood samples was collected at various times after HLH diagnosis to determine the concentration of eosinophils.

Time frame: 11 months

Mean Serum Concentration of Basophils

Blood samples was collected at various times after HLH diagnosis to determine the concentration of basophils.

Time frame: 11 months

Mean Plasma Concentration of D-Dimer

Blood samples was collected at various times after HLH diagnosis to determine the concentration of D-dimer.

Time frame: 11 months

Mean Plasma Concentration of Fibrinogen

Blood samples was collected at various times after HLH diagnosis to determine the concentration of fibrinogen.

Time frame: 11 months

Mean Activated Partial Thromboplastin Time (aPTT)

Blood samples was collected at various times after HLH diagnosis to determine the aPTT.

Time frame: 11 months

Mean Prothrombin Time

Blood samples was collected at various times after HLH diagnosis to determine the prothrombin time.

Time frame: 11 months

Mean Serum Concentration of C-Reactive Protein

Blood samples was collected at various times after HLH diagnosis to determine the concentration of c-reactive protein.

Time frame: 11 months

Mean Serum Concentration of Ferritin

Blood samples was collected at various times after HLH diagnosis to determine the concentration of ferritin.

Time frame: 11 months

Mean Plasma Concentration of Blood Urea Nitrogen

Blood samples was collected at various times after HLH diagnosis to determine the concentration of blood urea nitrogen.

Time frame: 11 months

Mean Serum Concentration of Creatinine

Blood samples was collected at various times after HLH diagnosis to determine the concentration of creatinine.

Time frame: 11 months

Mean Serum Concentration of Albumin

Blood samples was collected at various times after HLH diagnosis to determine the concentration of albumin.

Time frame: 11 months

Mean Serum Concentration of Sodium

Blood samples was collected at various times after HLH diagnosis to determine the concentration of sodium.

Time frame: 11 months

Mean Serum Concentration of Fasting Triglycerides

Blood samples was collected at various times after HLH diagnosis to determine the concentration of fasting triglycerides.

Time frame: 11 months

Mean Serum Concentration of Total Bilirubin

Blood samples was collected at various times after HLH diagnosis to determine the concentration of total bilirubin.

Time frame: 11 months

Mean Serum Concentration of Conjugated Bilirubin

Blood samples was collected at various times after HLH diagnosis to determine the concentration of conjugated bilirubin.

Time frame: 11 months

Mean Serum Concentration of Alanine Aminotransferase

Blood samples was collected at various times after HLH diagnosis to determine the concentration of alanine aminotransferase.

Time frame: 11 months

Mean Serum Concentration of Aspartate Aminotransferase

Blood samples was collected at various times after HLH diagnosis to determine the concentration of aspartate aminotransferase.

Time frame: 11 months

Mean Serum Concentration of Alkaline Phosphatase

Blood samples was collected at various times after HLH diagnosis to determine the concentration of alkaline phosphatase.

Time frame: 11 months

Mean Serum Concentration of Lactate Dehydrogenase

Blood samples was collected at various times after HLH diagnosis to determine the concentration of lactate dehydrogenase.

Time frame: 11 months

Mean Serum Concentration of Immunoglobulin G (IgG)

Blood samples was collected at various times after HLH diagnosis to determine the concentration of IgG.

Time frame: 11 months