Mixed cryoglobulinemia: demographic, clinical, and serologic features and survival in 231 patients
Section snippets
Methods
The study population (231 patients; 174 females, 57 males) was recruited between 1972 and 2001 from the Rheumatology Unit of the University of Pisa, Medical School (Table 4). At the time of diagnosis, all patients showed clinicoserologic and pathologic features sufficient for a diagnosis of definite MC according to preliminary classification criteria (16) (Table 3).
Clinical assessment was performed on the basis of 2 main categories: demographic data and clinical features observed at the time
Results
Demographic, clinicoserologic, and virologic features of MC patients at the beginning and at the end of the follow-up are summarized in Table 4. The patient mean age at the beginning of the follow-up was 56.4 ± 11.2 (SD) years. At the end of the study, 21 patients (9.1%) were lost to follow-up. During 2001, the vital status of the remaining 210 patients was ascertained: 113 of 210 (54%) were still alive, whereas 97 of 210 (46%) were deceased after 12.3 ± 7.6 (mean ± SD) years from disease
Discussion
The present study evaluated the prognostic value of epidemiologic and clinicoserologic parameters and survival in a large series of patients with MC. Renal failure caused by chronic glomerulonephritis was responsible for death in one third of deceased patients; chronic hepatitis with cirrhosis, widespread vasculitis, and B-cell non-Hodgkin’s lymphoma were other important causes of death. The cumulative 10-year survival calculated by the Kaplan-Meier method showed a significantly lower rate in
Clodoveo Ferri, MD: Professor of Rheumatology, Department of Internal Medicine, University of Pisa, Medical School, Pisa, Italy
References (108)
- et al.
Atypical glycosilation of an IgG monoclonal cryoimmunoglobulin
J Biol Chem
(1987) - et al.
Biologic and clinical significance of cryoglobulins
Am J Med
(1974) - et al.
Mixed cryoglobulinemiaclinical aspects and long-term follow-up of 40 patients
Am J Med
(1980) - et al.
Cryoglobulinemia. A clinical and laboratory study. II. Cryoglobulins with rheumatoid factor activity
Am J Med
(1966) - et al.
Two-dimensional polyacrylamide gel electrophoresis analysis of cryoglobulins and identification of an IgM-associated peptide
J Immunol Methods
(1994) - et al.
Detection and distribution of hepatitis C—related proteins in lymph nodes of patients with type II cryoglobulinemia and neoplastic or non-neoplastic lymphoproliferation
Blood
(1996) - et al.
Cryoglobulinemia in chronic liver diseasesRole of hepatitis C virus and liver damage
Gastroenterology
(1994) - et al.
Low-grade malignant lymphoma, hepatitis C virus infection, and mixed cryoglobulinemia
Blood
(1994) - et al.
Infection of peripheral blood mononuclear cells by hepatitis C virus in mixed cryoglobulinemia
Blood
(1993) - et al.
Infection of peripheral mononuclear blood cells by hepatitis C virus
J Hepatol
(1992)
Antibodies to hepatitis C virus in autoimmune liver diseaseEvidence for geographical heterogeneity
Lancet
Haplotype HLA-B8-DR3 confers susceptibility to hepatitis C virus—related mixed cryoglobulinemia
Blood
A revised European-American classification of lymphoid neoplasmsA proposal from the International Lymphoma Study Group
Blood
Does hepatitis C virus cause severe liver disease only in people who drink alcohol?
Lancet Infect Dis
Alpha-interferon in mixed cryoglobulinemia patientsA randomized crossover controlled trial
Blood
Renal involvement in hepatitis C infectionCryoglobulinemic glomerulonephritis
Kidney Int
Lung involvement in essential mixed cryoglobulinemia
Am J Med
Bcl-2 rearrangement in patients with chronic hepatitis C associated with essential mixed cryoglobulinemia type II
Blood
Long-term results of therapy with interferon-alpha for type II essential mixed cryoglobulinemia
Blood
Cryoglobulinemia based on interaction between a gamma macroglobulin and 7S gamma globulin
Am J Med
Hyperproteinemia associated with multiple myeloma. With report of a case in which an extraordinary hyperproteinemia was associated with trombosis of the retinal veins and symptoms suggesting Raynaud’s disease
Bull Johns Hopkins Hosp
Cryoglobulinemia induced by monoclonal immunoglobulin G rheumatoid factor derived from autoimmune MRL/MpJ-lpr/lpr mice
J Immunol
Anti-DNA antibodies from autoimmune mice arise by clonal expansion and somatic mutation
J Exp Med
Abnormal glycosilation of serum IgG from patients with chronic inflammatory diseases
Arthritis Rheum
Studies on monoclonal antibodies. II. Immune complex (IgM-IgG) cryoglobulinemiathe mechanism of cryoprecipitation
J Immunol
Type II mixed cryoglobulinemia is a non-malignant B-cell disorder. Clinical and molecular findings do not support bone marrow pathologic diagnosis of low grade B-cell lymphoma
Arthritis Rheum
Clonality of B-cells in portal lymphoid infiltrates of HCV-infected livers
J. Pathol
Hepatitis C virus infection in type II mixed cryoglobulinemia (review)
Arthritis Rheum
Blood viscosity and filtration abnormalities in mixed cryoglobulinemia patients
Clin Exp Rheumatol
Cryoglobulins (review)
J Clin Pathol
Mixed cryoglobulinemia cross-reactive idiotypesImplication for relationship of MC to rheumatic and lymphoproliferative diseases
Semin Hematol
The cryoglobulinsAn overview
Eur J Clin Invest
Evaluation of cryoglobulins
Arch Pathol Lab Med
Cryoglobulinemic vasculitis (review)
Arthritis Rheum
Essential mixed cryoglobulinemia, type IIA manifestation of low malignant lymphoma? Clinical-morphological study of 12 cases with special reference to immunohistochemical findings in liver frozen sections
Acta Haematol
CryoglobulinaemiasA multi-centre study of the early clinical and laboratory manifestations of primary and secondary disease
QJM
Nomenclature of systemic vasculitidesProposal of an international consensus conference
Arthritis Rheum
Hepatitis C virus and mixed cryoglobulinemia (review)
Eur J Clin Invest
Antibodies to hepatitis C virus in patients with mixed cryoglobulinemia
Arthritis Rheum
Hepatitis CA multifaceted disease. Review of extrahepatic manifestations (review)
Ann Intern Med
Isolation of a cDNA clone derived from a blood-borne non-A non-B viral hepatitis genome
Science
Hepatitis C virus infection
N Engl J Med
Localization of hepatitis C virus in cutaneous vasculitic lesions in patients with type II cryoglobulinemia
Arthritis Rheum
Hepatitis C virus, B-cell disorders, and non-Hodgkin’s lymphoma
Immunological disorders in C virus chronic active hepatitisA prospective case-control study
Hepatology
Hepatitis CAn epidemiological review
J Viral Hepat
Thyroid cancer in patients with hepatitis C infection
JAMA
Mixed cryoglobulinemiaA cross-road between autoimmune and lymphoproliferative disorders
Lupus
The lymphoid system in hepatitis C virus infectionAutoimmunity, mixed cryoglobulinemia, and overt B-cell malignancy
Semin Liver Dis
Bcl-2A repressor of lymphocyte death
Immunol Today
Cited by (434)
Headaches and Vasculitis
2024, Neurologic ClinicsVasculitides and Hepatitis C Infection
2024, Infection and Autoimmunity
Clodoveo Ferri, MD: Professor of Rheumatology, Department of Internal Medicine, University of Pisa, Medical School, Pisa, Italy
Marco Sebastiani, MD: Fellow of Rheumatology, Department of Internal Medicine, University of Pisa, Medical School, Pisa, Italy
Dilia Giuggioli, MD: Assistant Professor, Department of Internal Medicine, University of Pisa, Medical School, Pisa, Italy
Massimimiliano Cazzato, MD: Assistant Professor, Department of Internal Medicine, University of Pisa, Medical School, Pisa, Italy
Giovanni Longombardo, BS: Investigator, Department of Internal Medicine, University of Pisa, Medical School, Pisa, Italy
Alessandro Antonelli, MD: Investigator, Department of Internal Medicine, University of Pisa, Medical School, Pisa, Italy
Rodolfo Puccini, MD: Assistant Professor, Division of Nephrology, S. Chiara Hospital, Pisa, Italy
Claudio Michelassi, BS: Investigator, Clinical Physiology, CNR Pisa, Italy
Anna Linda Zignego, MD: Associate Professor of Internal Medicine, Department of Internal Medicine, University of Florence, Medical School, Florence, Italy.