Survival and Prognostic Factors in Mixed Cryoglobulinemia: Data from 246 Cases
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Le résumé fourni par la source
Introduction: The clinical and therapeutic management of mixed cryoglobulinemia (MC) remains a subject of controversy. In addition, most studies have not recorded the long-term follow-up and the outcome of these cases. Material and Methods: We enrolled 246 patients affected by MC who were consecutively admitted to our Department from January 1993 to February 2013. Clinical and biological data had been recorded until June 2014. Results: The median age (at diagnosis) was 60 years (range 26–83). The aetiology was HCV in 95% of patients, HBV in 3% and “essential” in 2%. HCV genotype was 1b in 57%, genotypes 2–3 in 43%. MC was Type II in 203 of the cases (87%) and Type III in 52 (13%). The most frequent clinical manifestations were purpura (72%), chronic liver disease (70%), glomerulonephritis (35%), arthralgias (58%), peripheral neuropathy (21%), non-Hodgkin lymphoma (15%) and cutaneous ulcers (3%). Purpura, arthralgias, peripheral neuropathy, glomerulonephritis and non-Hodgkin lymphoma were more frequently observed in Type II than in Type III MC (p < 0.05). Treatments were interferon (IFN) or Pegilated-IFN (PEG-IFN) alone or plus Ribavirin (RIBA) in 101 cases, steroids with or without alkylating agents in 33 cases, Rituximab in 8 patients. The complete clinical, virological and immunological responses were associated with PEG-IFN plus RIBA. Severe infections were associated with renal failure. At 10 years, the overall survival rate was 71% in Type II MC and 84% in Type III (p < 0.053). Conclusions: From our data, antiviral therapy is the first-line therapy in HCV-related MC, whereas steroids, alkylating agents and Rituximab should be considered as a second-line therapy. Given the heterogeneity of the disease, the role of these different therapeutic strategies should be checked in randomized controlled trials.
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Le contrôle bibliographique ouvert
DOI retrouvé dans Crossref DOI retrouvé ; titre concordant.
- Titre Crossref
- Survival and Prognostic Factors in Mixed Cryoglobulinemia: Data from 246 Cases
- Date Crossref
- 03/05/2018
- Éditeur
- MDPI AG
- Type
- journal-article
Ce recoupement confirme des métadonnées liées au DOI. Il ne confirme ni la méthode ni les conclusions de l’étude, et il ne compte pas comme une seconde source scientifique indépendante.
Où se fait cette recherche
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Centro di Riferimento Oncologico pays non établi dans la noticeÉtablissement de santé
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Istituti di Ricovero e Cura a Carattere Scientifico pays non établi dans la noticeÉtablissement de santé
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University of Trieste Department of Clinical and Surgical Sciences pays non établi dans la noticeUniversité ou école supérieure
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Clinical and Experimental Onco-Haematology Unit pays non établi dans la noticeÉtablissement de santé
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CRO Aviano National Cancer Institute IRCCS Epidemiology and Statistic Unit pays non établi dans la noticeStructure de recherche
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Pordenone General Hospital Department of Internal Medicine pays non établi dans la noticeÉtablissement de santé
Centro di Riferimento Oncologico, Istituti di Ricovero e Cura a Carattere Scientifico et Department of Clinical and Surgical Sciences — University of Trieste, avec 3 autres affiliations.
Une affiliation ne permet pas de déduire la nationalité d’un auteur.