Glycosylated Ferritin in Macrophagic Activation Syndromes

The objectives of this study are: 1. . To evaluate the diagnostic performance of the assay of the glycosylated ferritin under HLH syndrome and Still's disease in adults. 2. . To study the different glycoforms of ferritin from serum of patient with hyperferritinemia. Materials and methods 1. Patient groups studied and control group * Any suspicion of HLH or Still's disease syndrome in adults * Control groups: sepsis, severe sepsis, septic shock, inflammation ( lupus, rheumatoid arthritis), severe hepatic necrosis, hematologic malignancy at diagnosis or relapse 2 . Samples * Serum will be collected for the determination of glycosylated ferritin in patients with suspicion of HLH syndrome or Still's disease in adults * For the orther patients remaining serum samples will be collected from ferritin sample of the routine biological evaluation in patients with infammatory diseases ( lupus, rheumatoid arthritis) , hematologic malignancies at diagnosis or relapse, hepatic necrosis or sepsis. 3 . Characterization of patients based on diagnostic criteria (clinical and biological) retained (with collaborating physicians). After approval of the EC CHU Brugmann, the protocol will be submitted to other EC institutions. 4 . Analysis of the glycosylation profile of ferritin by mass spectrometry. (in collaboration with the Faculty of Pharmacy , Department of Pharmaceutical Chemistry , Van Antwerpen P and Delporte C) Analysis and interpretation 1. . Determination of the sensitivity and specificity of the diagnostic assay of the glycosylated ferritin under HLH syndrome and Still's disease . 2. . Characterization of subgroups of patients according to the following information: Ferritin and its glycosylated fraction, documented infections , fever, MOF , hepatomegaly, splenomegaly, haemophagocytosis (when bone marrow aspirate available), thrombocytosis, anemia, leukopenia, neutropenia, liver enzymes, hypertriglyceridemia, coagulopathy, hypofibrinogenemia, VS / CRP. 3. . Analysis of glycosylation patterns according to the patient subgroup.