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CRYOGLOBULINEMIA - 08/09/11

Doi : 10.1016/S0889-8588(05)70129-5 
Angela Dispenzieri, MD a, Peter D. Gorevic, MD b
a Division of Hematology and Internal Medicine, Mayo Clinic and Mayo Foundation, Rochester, Minnesota (AD) 
b Department of Medicine, The Mount Sinai Medical Center, New York, New York (PDG) 

Riassunto

Wintrobe and Buell228 first described reversible cryoprecipitability of serum in a patient with multiple myeloma in 1933. Lerner systematically studied cold-induced precipitation of these immunoglobulins, and in 1947 Lerner and Watson128 first employed the term cryoglobulins. In the 1960s, the clinical studies of Meltzer and others146, 147 delineated a distinct syndrome of purpura, arthralgias, asthenia, and renal disease–often in the context of immune complex deposition or vasculitis. By 1974, Brouet et al17 popularized a system which classified cryoglobulinemia by the components of the cryoprecipitate: type I, isolated monoclonal immunoglobulins; type II, a monoclonal component, usually immunoglobulin M (IgM), possessing activity toward polyclonal immunoglobulins, usually immunoglobulin G (IgG); and type III, polyclonal immunoglobulins of more than one isotype. This classification provided a framework for clinical correlations.

Associated conditions, such as lymphoproliferative disorders, connective tissue disorders, infection, liver disease and others listed in the box on the following page, were observed in some92, 105, 199, 218, 225 but not all patients.

In several large series, 34% to 71% of cases of cryoglobulinemia were not associated with other disease states; these were termed essential or primary cryoglobulinemia (Table 1).17, 92, 158 Following its identification in the late 1980s, the hepatitis C virus (HCV) was recognized to be a potential causative factor for essential mixed cryoglobulinemia (EMC).66, 174 This association has been confirmed by a number of groups since its original description in 1990. The mechanism of HCV involvement in mixed cryoglobulinemia (MC) and its potential role in the development of other autoimmune diseases and non-Hodgkin's

Cryoglobulinemia: Clinical and Experimental Associations90

Infections
Viral
Epstein-Barr virus
Cytomegalovirus
Hepatitis B virus
Hepatitis A virus
Adenovirus
Hepatitis C virus65, 174
HIV7, 54, 161
Bacterial
Subacute bacterial endocarditis (with or without nephritis)
Lepromatous leprosy (with or without erythema nodosum)
Acute poststreptococcal nephritis
Syphilis
Lyme disease (with or without erythema chronicum migrans)
Postintestinal bypass with arthritis
Q fever216
Fungal
Coccidioidomycosis
Parasitic
Kala-azar
Toxoplasmosis
Tropical splenomegaly syndrome
Echinococcosis
Malaria
Schistosomiasis
Trypanosomiasis
Autoimmune diseases
Systemic lupus erythematosus
Nephritis, hypocomplementemia
Drug-induced lupus (procainamide)
Rheumatoid arthritis
Polyarteritis nodosa (HBsAg positive and negative)
Kawasaki syndrome (with or without macroglobulinemia)
Sjögren's syndrome
Scleroderma
Sarcoidosis
Thyroiditis
Henoch-Schönlein purpura
Behçet's syndrome
Polymyositis
Inflammatory bowel disease
Celiac disease, ulcerative colitis, regional enteritis
Endomyocardial fibrosis
Pulmonary fibrosis
Cutaneous vasculitis
Pemphigus vulgaris
Erythema elevatum diutinum
Cold-induced urticaria
Epidermolysis bullosa acquisita
Erythema multiforme
POEMS syndrome
Pyoderma gangrenosum
Lymphoproliferative disorders
Macroglobulinemia (primary and secondary)17, 92, 107, 173
Lymphoma (Hodgkin's and non-Hodgkin's)17, 92, 107, 173
Chronic lymphocytic leukemia
Immunoblastic lymphadenopathy
Hairy cell leukemia
Renal diseases
Proliferative glomerulonephritis
Liver diseases
Cirrhosis (Laënnec's, postnecrotic)
Biliary cirrhosis
Chronic hepatitis
Familial (symptomatic, asymptomatic)
Essential
Experimental
Pneumococcal vaccines
Streptococcal (A and C) hyperimmunization
New Zealand black/New Zealand white, MRL/1, BXSB mice
lymphoma are now areas of active investigation. This article summarizes current information concerning the pathogenesis, clinical and laboratory features, and treatment of cryoglobulinemia, with an emphasis on relationships between the occurrence of cryoglobulins in HCV infection and lymphoproliferative disorders.

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Mappa


 Address reprint requests to Angela Dispenzieri, MD, Division of Hematology and Internal Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN 55905
Supported in part by Grant CA62242 from the National Institutes of Health.


© 1999  W. B. Saunders Company. Pubblicato da Elsevier Masson SAS. Tutti i diritti riservati.
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Vol 13 - N° 6

P. 1315-1349 - dicembre 1999 Ritorno al numero
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  • PROGNOSTIC FACTORS IN MULTIPLE MYELOMA
  • S. Vincent Rajkumar, Philip R. Greipp
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  • WALDENSTRÖM'S MACROGLOBULINEMIA
  • Meletios A. Dimopoulos, Eleni Galani, Charis Matsouka

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