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

Cesare Mazzaro; Luigino Dal Maso; Endri Mauro; Valter Gattei; Michela Ghersetti; Pietro Bulian; Giulia Moratelli; Gabriele Grassi; Francesca Zorat; Gabriele Pozzato

doi:10.3390/diseases6020035

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

Cesare Mazzaro, Luigino Dal Maso, Endri Mauro, Valter Gattei, Michela Ghersetti, Pietro Bulian, Giulia Moratelli, Gabriele Grassi, Francesca Zorat, Gabriele Pozzato

Centro di Riferimento Oncologico

Research output: Contribution to journal › Article › peer-review

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.

Original language	English
Journal	Rare Diseases
Volume	6
Issue number	2
DOIs	https://doi.org/10.3390/diseases6020035
Publication status	Published - May 3 2018

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title = "Survival and Prognostic Factors in Mixed Cryoglobulinemia: Data from 246 Cases",

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.",

author = "Cesare Mazzaro and Maso, {Luigino Dal} and Endri Mauro and Valter Gattei and Michela Ghersetti and Pietro Bulian and Giulia Moratelli and Gabriele Grassi and Francesca Zorat and Gabriele Pozzato",

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doi = "10.3390/diseases6020035",

language = "English",

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TY - JOUR

T1 - Survival and Prognostic Factors in Mixed Cryoglobulinemia

T2 - Data from 246 Cases

AU - Mazzaro, Cesare

AU - Maso, Luigino Dal

AU - Mauro, Endri

AU - Gattei, Valter

AU - Ghersetti, Michela

AU - Bulian, Pietro

AU - Moratelli, Giulia

AU - Grassi, Gabriele

AU - Zorat, Francesca

AU - Pozzato, Gabriele

PY - 2018/5/3

Y1 - 2018/5/3

N2 - 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.

AB - 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.

U2 - 10.3390/diseases6020035

DO - 10.3390/diseases6020035

M3 - Article

C2 - 29751499

SN - 2167-549X

VL - 6

JO - Rare Diseases

JF - Rare Diseases

IS - 2

ER -

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

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