Increased level of H-ferritin and its imbalance with L-ferritin, in bone marrow and liver of patients with adult onset Still's disease, developing macrophage activation syndrome, correlate with the severity of the disease

Giovanni Triolo, Francesco Ciccia, Roberto Giacomelli, Paola Cipriani, Vasiliki Liakouli, Aroldo Rizzo, Francesco Carubbi, Paola Di Benedetto, Piero Ruscitti, Onorina Berardicurti

    Risultato della ricerca: Article

    26 Citazioni (Scopus)

    Abstract

    In this paper, we aimed to evaluate the levels of ferritin enriched in H subunits (H-ferritin) and ferritin enriched in L subunits (L-ferritin) and the cells expressing these 2 molecules, in the bone marrow (BM) and liver biopsies obtained from adult onset Still's disease (AOSD) patients who developed macrophage activation syndrome (MAS), and correlating these data with the severity of the disease.Twenty-one patients with MAS-associated AOSD underwent BM biopsy and among them, 9 patients with hepatomegaly and elevated liver enzymes underwent liver biopsy. All the samples were stained by both immunohistochemistry and immunofluorescence. A statistical analysis was performed to estimate the possible correlation among both H-ferritin and L-ferritin tissue expression and the clinical picture of the disease. Furthermore, the same analysis was performed to evaluate the possible correlation among the number of CD68/H-ferritin or CD68/L-ferritin positive cells and the clinical picture.Both immunohistochemical and immunofluorescence analysis demonstrated an increased tissue H-ferritin expression, in the BM and liver samples of our patients. This increased expression correlated with the severity of the disease. An inflammatory infiltrate, enriched in CD68 macrophages, expressing H-ferritin was observed in both the BM and the liver samples of our patients. Furthermore, we observed, that this increased number of CD68/H-ferritin positive cells significantly correlated with the severity of clinical picture and this specific BM infiltrate correlated with the mortality rate, reported in our cohort.Our data showed an imbalance between the levels of H- and L-ferritin in different organs of patients with MAS-associated AOSD and the evidence of a strong infiltrate of CD68/H-ferritin positive cells in the same organs. Furthermore, a strong correlation among both the tissue H-ferritin and the CD68/H-ferritin positive cells and the clinical picture was observed.
    Lingua originaleEnglish
    pagine (da-a)429-437
    Numero di pagine9
    RivistaAutoimmunity Reviews
    Volume14
    Stato di pubblicazionePublished - 2015

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    Macrophage Activation Syndrome
    Adult-Onset Still's Disease
    Apoferritins
    Bone Marrow
    Liver
    Ferritins
    Biopsy
    Fluorescent Antibody Technique
    Hepatomegaly

    All Science Journal Classification (ASJC) codes

    • Immunology and Allergy
    • Immunology

    Cita questo

    Increased level of H-ferritin and its imbalance with L-ferritin, in bone marrow and liver of patients with adult onset Still's disease, developing macrophage activation syndrome, correlate with the severity of the disease. / Triolo, Giovanni; Ciccia, Francesco; Giacomelli, Roberto; Cipriani, Paola; Liakouli, Vasiliki; Rizzo, Aroldo; Carubbi, Francesco; Di Benedetto, Paola; Ruscitti, Piero; Berardicurti, Onorina.

    In: Autoimmunity Reviews, Vol. 14, 2015, pag. 429-437.

    Risultato della ricerca: Article

    Triolo, Giovanni ; Ciccia, Francesco ; Giacomelli, Roberto ; Cipriani, Paola ; Liakouli, Vasiliki ; Rizzo, Aroldo ; Carubbi, Francesco ; Di Benedetto, Paola ; Ruscitti, Piero ; Berardicurti, Onorina. / Increased level of H-ferritin and its imbalance with L-ferritin, in bone marrow and liver of patients with adult onset Still's disease, developing macrophage activation syndrome, correlate with the severity of the disease. In: Autoimmunity Reviews. 2015 ; Vol. 14. pagg. 429-437.
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    title = "Increased level of H-ferritin and its imbalance with L-ferritin, in bone marrow and liver of patients with adult onset Still's disease, developing macrophage activation syndrome, correlate with the severity of the disease",
    abstract = "In this paper, we aimed to evaluate the levels of ferritin enriched in H subunits (H-ferritin) and ferritin enriched in L subunits (L-ferritin) and the cells expressing these 2 molecules, in the bone marrow (BM) and liver biopsies obtained from adult onset Still's disease (AOSD) patients who developed macrophage activation syndrome (MAS), and correlating these data with the severity of the disease.Twenty-one patients with MAS-associated AOSD underwent BM biopsy and among them, 9 patients with hepatomegaly and elevated liver enzymes underwent liver biopsy. All the samples were stained by both immunohistochemistry and immunofluorescence. A statistical analysis was performed to estimate the possible correlation among both H-ferritin and L-ferritin tissue expression and the clinical picture of the disease. Furthermore, the same analysis was performed to evaluate the possible correlation among the number of CD68/H-ferritin or CD68/L-ferritin positive cells and the clinical picture.Both immunohistochemical and immunofluorescence analysis demonstrated an increased tissue H-ferritin expression, in the BM and liver samples of our patients. This increased expression correlated with the severity of the disease. An inflammatory infiltrate, enriched in CD68 macrophages, expressing H-ferritin was observed in both the BM and the liver samples of our patients. Furthermore, we observed, that this increased number of CD68/H-ferritin positive cells significantly correlated with the severity of clinical picture and this specific BM infiltrate correlated with the mortality rate, reported in our cohort.Our data showed an imbalance between the levels of H- and L-ferritin in different organs of patients with MAS-associated AOSD and the evidence of a strong infiltrate of CD68/H-ferritin positive cells in the same organs. Furthermore, a strong correlation among both the tissue H-ferritin and the CD68/H-ferritin positive cells and the clinical picture was observed.",
    keywords = "Adult-onset Still's disease; Hyperferritinemia; Macrophage activation syndrome; Adult; Age of Onset; Animals; Apoferritins; Bone Marrow; Humans; Liver; Macrophage Activation Syndrome; Still's Disease, Adult-Onset; Immunology; Immunology and Allergy; Medicine (all)",
    author = "Giovanni Triolo and Francesco Ciccia and Roberto Giacomelli and Paola Cipriani and Vasiliki Liakouli and Aroldo Rizzo and Francesco Carubbi and {Di Benedetto}, Paola and Piero Ruscitti and Onorina Berardicurti",
    year = "2015",
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    pages = "429--437",
    journal = "Autoimmunity Reviews",
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    TY - JOUR

    T1 - Increased level of H-ferritin and its imbalance with L-ferritin, in bone marrow and liver of patients with adult onset Still's disease, developing macrophage activation syndrome, correlate with the severity of the disease

    AU - Triolo, Giovanni

    AU - Ciccia, Francesco

    AU - Giacomelli, Roberto

    AU - Cipriani, Paola

    AU - Liakouli, Vasiliki

    AU - Rizzo, Aroldo

    AU - Carubbi, Francesco

    AU - Di Benedetto, Paola

    AU - Ruscitti, Piero

    AU - Berardicurti, Onorina

    PY - 2015

    Y1 - 2015

    N2 - In this paper, we aimed to evaluate the levels of ferritin enriched in H subunits (H-ferritin) and ferritin enriched in L subunits (L-ferritin) and the cells expressing these 2 molecules, in the bone marrow (BM) and liver biopsies obtained from adult onset Still's disease (AOSD) patients who developed macrophage activation syndrome (MAS), and correlating these data with the severity of the disease.Twenty-one patients with MAS-associated AOSD underwent BM biopsy and among them, 9 patients with hepatomegaly and elevated liver enzymes underwent liver biopsy. All the samples were stained by both immunohistochemistry and immunofluorescence. A statistical analysis was performed to estimate the possible correlation among both H-ferritin and L-ferritin tissue expression and the clinical picture of the disease. Furthermore, the same analysis was performed to evaluate the possible correlation among the number of CD68/H-ferritin or CD68/L-ferritin positive cells and the clinical picture.Both immunohistochemical and immunofluorescence analysis demonstrated an increased tissue H-ferritin expression, in the BM and liver samples of our patients. This increased expression correlated with the severity of the disease. An inflammatory infiltrate, enriched in CD68 macrophages, expressing H-ferritin was observed in both the BM and the liver samples of our patients. Furthermore, we observed, that this increased number of CD68/H-ferritin positive cells significantly correlated with the severity of clinical picture and this specific BM infiltrate correlated with the mortality rate, reported in our cohort.Our data showed an imbalance between the levels of H- and L-ferritin in different organs of patients with MAS-associated AOSD and the evidence of a strong infiltrate of CD68/H-ferritin positive cells in the same organs. Furthermore, a strong correlation among both the tissue H-ferritin and the CD68/H-ferritin positive cells and the clinical picture was observed.

    AB - In this paper, we aimed to evaluate the levels of ferritin enriched in H subunits (H-ferritin) and ferritin enriched in L subunits (L-ferritin) and the cells expressing these 2 molecules, in the bone marrow (BM) and liver biopsies obtained from adult onset Still's disease (AOSD) patients who developed macrophage activation syndrome (MAS), and correlating these data with the severity of the disease.Twenty-one patients with MAS-associated AOSD underwent BM biopsy and among them, 9 patients with hepatomegaly and elevated liver enzymes underwent liver biopsy. All the samples were stained by both immunohistochemistry and immunofluorescence. A statistical analysis was performed to estimate the possible correlation among both H-ferritin and L-ferritin tissue expression and the clinical picture of the disease. Furthermore, the same analysis was performed to evaluate the possible correlation among the number of CD68/H-ferritin or CD68/L-ferritin positive cells and the clinical picture.Both immunohistochemical and immunofluorescence analysis demonstrated an increased tissue H-ferritin expression, in the BM and liver samples of our patients. This increased expression correlated with the severity of the disease. An inflammatory infiltrate, enriched in CD68 macrophages, expressing H-ferritin was observed in both the BM and the liver samples of our patients. Furthermore, we observed, that this increased number of CD68/H-ferritin positive cells significantly correlated with the severity of clinical picture and this specific BM infiltrate correlated with the mortality rate, reported in our cohort.Our data showed an imbalance between the levels of H- and L-ferritin in different organs of patients with MAS-associated AOSD and the evidence of a strong infiltrate of CD68/H-ferritin positive cells in the same organs. Furthermore, a strong correlation among both the tissue H-ferritin and the CD68/H-ferritin positive cells and the clinical picture was observed.

    KW - Adult-onset Still's disease; Hyperferritinemia; Macrophage activation syndrome; Adult; Age of Onset; Animals; Apoferritins; Bone Marrow; Humans; Liver; Macrophage Activation Syndrome; Still's Disease, Adult-Onset; Immunology; Immunology and Allergy; Medic

    UR - http://hdl.handle.net/10447/206736

    UR - http://www.elsevier.com/locate/autrev

    M3 - Article

    VL - 14

    SP - 429

    EP - 437

    JO - Autoimmunity Reviews

    JF - Autoimmunity Reviews

    SN - 1568-9972

    ER -