Cryoglobulinemia

Angela Dispenzieri, Peter D. Gorevic

Research output: Contribution to journalArticle

95 Citations (Scopus)

Abstract

Cryoglobulinemia may be found in a spectrum of disorders spanning clearcut-B-cell neoplastic states, in which cryoprecipitation manifests as ischemic or occlusive vasculopathy, to a variety of immune complex diseases, in which vasculitis or glomerulonephritis may occur. Symptomatic cryoglobulinemia is many diseases, driven by and driving antibody-antigen responses, hepatic dysfunction, lymphoproliferation, and immune complexes. Distinguishing features that cause only some cryoglobulins to be symptomatic, elucidating the pathogenic mechanisms of HCV in cryoglobulin formation, and devising better therapies and more systematic evaluation of existing therapies are among the challenges for the future. Prognostication and classification will continue to rely on Brouet's classification (types I, II, and III), but additional features will probably include the presence or absence of HCV, HCV factors (genotype, titer), coexisting infections, B-cell clone burden, host factors, and immune system interactions (B- and T-cell idiotype networks, cytokines). Although antiviral therapy is a reasonable option for HCV-associated cryoglobulinemia, not all patients are HCV- positive, and only 60% to 80% of HCV-positive patients respond to IFN. In addition, not all patients tolerate IFN, and in those who do, the response is often short-lived once the treatment is discontinued. Only creative strategies, systematically studied, will provide long-awaited solutions.

Original languageEnglish (US)
Pages (from-to)1315-1349
Number of pages35
JournalHematology/Oncology Clinics of North America
Volume13
Issue number6
StatePublished - 1999

Fingerprint

Cryoglobulinemia
Cryoglobulins
B-Lymphocytes
Immune Complex Diseases
Therapeutics
Glomerulonephritis
Vasculitis
Antigen-Antibody Complex
Antibody Formation
Antiviral Agents
Immune System
Clone Cells
Genotype
Cytokines
T-Lymphocytes
Antigens
Liver
Infection

ASJC Scopus subject areas

  • Hematology
  • Oncology

Cite this

Cryoglobulinemia. / Dispenzieri, Angela; Gorevic, Peter D.

In: Hematology/Oncology Clinics of North America, Vol. 13, No. 6, 1999, p. 1315-1349.

Research output: Contribution to journalArticle

Dispenzieri, A & Gorevic, PD 1999, 'Cryoglobulinemia', Hematology/Oncology Clinics of North America, vol. 13, no. 6, pp. 1315-1349.
Dispenzieri, Angela ; Gorevic, Peter D. / Cryoglobulinemia. In: Hematology/Oncology Clinics of North America. 1999 ; Vol. 13, No. 6. pp. 1315-1349.
@article{b5b81a1ca02d483a878214893068fa4a,
title = "Cryoglobulinemia",
abstract = "Cryoglobulinemia may be found in a spectrum of disorders spanning clearcut-B-cell neoplastic states, in which cryoprecipitation manifests as ischemic or occlusive vasculopathy, to a variety of immune complex diseases, in which vasculitis or glomerulonephritis may occur. Symptomatic cryoglobulinemia is many diseases, driven by and driving antibody-antigen responses, hepatic dysfunction, lymphoproliferation, and immune complexes. Distinguishing features that cause only some cryoglobulins to be symptomatic, elucidating the pathogenic mechanisms of HCV in cryoglobulin formation, and devising better therapies and more systematic evaluation of existing therapies are among the challenges for the future. Prognostication and classification will continue to rely on Brouet's classification (types I, II, and III), but additional features will probably include the presence or absence of HCV, HCV factors (genotype, titer), coexisting infections, B-cell clone burden, host factors, and immune system interactions (B- and T-cell idiotype networks, cytokines). Although antiviral therapy is a reasonable option for HCV-associated cryoglobulinemia, not all patients are HCV- positive, and only 60{\%} to 80{\%} of HCV-positive patients respond to IFN. In addition, not all patients tolerate IFN, and in those who do, the response is often short-lived once the treatment is discontinued. Only creative strategies, systematically studied, will provide long-awaited solutions.",
author = "Angela Dispenzieri and Gorevic, {Peter D.}",
year = "1999",
language = "English (US)",
volume = "13",
pages = "1315--1349",
journal = "Hematology/Oncology Clinics of North America",
issn = "0889-8588",
publisher = "W.B. Saunders Ltd",
number = "6",

}

TY - JOUR

T1 - Cryoglobulinemia

AU - Dispenzieri, Angela

AU - Gorevic, Peter D.

PY - 1999

Y1 - 1999

N2 - Cryoglobulinemia may be found in a spectrum of disorders spanning clearcut-B-cell neoplastic states, in which cryoprecipitation manifests as ischemic or occlusive vasculopathy, to a variety of immune complex diseases, in which vasculitis or glomerulonephritis may occur. Symptomatic cryoglobulinemia is many diseases, driven by and driving antibody-antigen responses, hepatic dysfunction, lymphoproliferation, and immune complexes. Distinguishing features that cause only some cryoglobulins to be symptomatic, elucidating the pathogenic mechanisms of HCV in cryoglobulin formation, and devising better therapies and more systematic evaluation of existing therapies are among the challenges for the future. Prognostication and classification will continue to rely on Brouet's classification (types I, II, and III), but additional features will probably include the presence or absence of HCV, HCV factors (genotype, titer), coexisting infections, B-cell clone burden, host factors, and immune system interactions (B- and T-cell idiotype networks, cytokines). Although antiviral therapy is a reasonable option for HCV-associated cryoglobulinemia, not all patients are HCV- positive, and only 60% to 80% of HCV-positive patients respond to IFN. In addition, not all patients tolerate IFN, and in those who do, the response is often short-lived once the treatment is discontinued. Only creative strategies, systematically studied, will provide long-awaited solutions.

AB - Cryoglobulinemia may be found in a spectrum of disorders spanning clearcut-B-cell neoplastic states, in which cryoprecipitation manifests as ischemic or occlusive vasculopathy, to a variety of immune complex diseases, in which vasculitis or glomerulonephritis may occur. Symptomatic cryoglobulinemia is many diseases, driven by and driving antibody-antigen responses, hepatic dysfunction, lymphoproliferation, and immune complexes. Distinguishing features that cause only some cryoglobulins to be symptomatic, elucidating the pathogenic mechanisms of HCV in cryoglobulin formation, and devising better therapies and more systematic evaluation of existing therapies are among the challenges for the future. Prognostication and classification will continue to rely on Brouet's classification (types I, II, and III), but additional features will probably include the presence or absence of HCV, HCV factors (genotype, titer), coexisting infections, B-cell clone burden, host factors, and immune system interactions (B- and T-cell idiotype networks, cytokines). Although antiviral therapy is a reasonable option for HCV-associated cryoglobulinemia, not all patients are HCV- positive, and only 60% to 80% of HCV-positive patients respond to IFN. In addition, not all patients tolerate IFN, and in those who do, the response is often short-lived once the treatment is discontinued. Only creative strategies, systematically studied, will provide long-awaited solutions.

UR - http://www.scopus.com/inward/record.url?scp=0033377360&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0033377360&partnerID=8YFLogxK

M3 - Article

C2 - 10626153

AN - SCOPUS:0033377360

VL - 13

SP - 1315

EP - 1349

JO - Hematology/Oncology Clinics of North America

JF - Hematology/Oncology Clinics of North America

SN - 0889-8588

IS - 6

ER -