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Diseases 2018, 6(2), 35; https://doi.org/10.3390/diseases6020035

Survival and Prognostic Factors in Mixed Cryoglobulinemia: Data from 246 Cases

1
Clinical and Experimental Onco-Haematology Unit, CRO Aviano National Cancer Institute IRCCS, 33081 Aviano, Italy
2
Epidemiology and Statistic Unit, CRO Aviano National Cancer Institute IRCCS, 33081 Aviano, Italy
3
Department of Internal Medicine, Pordenone General Hospital, 33170 Pordenone, Italy
4
Department of Clinical and Surgical Sciences, University of Trieste, 34121 Trieste, Italy
5
Department of Life Sciences, University of Trieste, 34121 Trieste, Italy
*
Author to whom correspondence should be addressed.
Received: 31 March 2018 / Revised: 24 April 2018 / Accepted: 27 April 2018 / Published: 3 May 2018
(This article belongs to the Special Issue Hepatitis and Treatment)
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Abstract

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. View Full-Text
Keywords: hepatitis C virus; mixed cryoglobulinemia; interferon alpha; steroids; rituximab hepatitis C virus; mixed cryoglobulinemia; interferon alpha; steroids; rituximab
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Mazzaro, C.; Maso, L.D.; Mauro, E.; Gattei, V.; Ghersetti, M.; Bulian, P.; Moratelli, G.; Grassi, G.; Zorat, F.; Pozzato, G. Survival and Prognostic Factors in Mixed Cryoglobulinemia: Data from 246 Cases. Diseases 2018, 6, 35.

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