The clinical and immunochemical classification of 376 patients with cryoglobulinemia is presented. In 141 cases (37.5%) the syndrome was considered idiopathic. Among the secondary forms, chronic liver diseases and connective tissue diseases shared most frequently mixed cryoglobulins (18.8 and 15.4% of cases, respectively). Another common association was that with Waldenström's macroglobulinemia: cryoglobulins were found in 20% of these patients. In 5 patients (2 with the essential form and 3 with cryoglobulinemia secondary to Waldenström's disease) a Cl-inhibitor deficiency was discovered; the complement profile was characteristic of the acquired type and episodes of angioedema occurred. Studying the clinical course of 71 cases of essential mixed cryoglobulinemia followed-up for 4-20 years we concluded that the evolution of type II and type III cryoglobulinemias is different because that of type II seems to have a more severe course and may differentiate in a lymphoproliferative disorder; the immunochemical type of cryoglobulins seems therefore to have a prognostic significance. Because of the considerable overlap in the distribution of immunochemical types among the clinical subsets, a mixed classification (both biochemical and clinical) is proposed.

Adult↗

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T lymphocyte subpopulations defined by monoclonal antibodies in essential mixed cryoglobulinemia and in secondary cryoglobulinemias.

T lymphocyte subpopulations defined by monoclonal antibodies were determined in patients with essential mixed cryoglobulinemia (EMC) and secondary cryoglobulinemias (SC). A decrease of circulating lymphocytes and a reduction in the absolute number of T3+, T4+ and T8+ (p less than 0.01) as well as in the percentage of T4+ lymphocytes (p less than 0.05) were found in EMC. A significant decrease of T8+ cells, both in percentage (p less than 0.01) and absolute number (p less than 0.001), was evidenced in SC, while T3+ and T4+ cell counts were not significantly different from those of healthy controls.

Adult↗

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Cryoglobulins and infectious diseases.

The relationship between infectious diseases due to various pathogenetic factors and cryoglobulin production mechanisms has been investigated. Cryoglobulins have been evidenced in infections caused by very heterogeneous pathogens, i.e. leptospirosis, psittacosis, Mediterranean tick typhus, brucellosis, gram-negative bacterial septicemias, in which they had never been previously reported. In type A hepatitis a high cryoglobulin prevalence (91%) has been confirmed during the acute phase, with a rapid decrease both in prevalence and concentration in the subsequent stages of the disease. Cryoglobulins were all of type III and were mainly represented by IgM; anti-HAV-IgM antibodies have been evidenced in all but one cryoprecipitates. In non-A, non-B hepatitis a lower cryoglobulin prevalence (44.7%) was shown during the acute phase and the same fast decrease has been noted in the subsequent stages. Cryoglobulins were all of type III and in some cases polyclonal IgG was the only Ig class present in cryoprecipitates. The cryoglobulin prevalence in the acute phase of HBsAg-positive hepatitis amounted to 73.4%; all the cryoprecipitates were of type III. No correlation between the presence of cryoglobulins and HBeAg positivity or between cryoglobulins and delta agent infections was found. In all the cases studied the presence of cryoglobulins was related to the persistence of liver damage. Cryoglobulins were not found in HBsAg chronic carriers, while they have been evidenced, by a preliminary study, in 41.6% of HTLV-III antibody-positive subjects complaining of a persistent generalized lymphadenopathy without clinical or laboratory signs of liver impairment. No HTLV-III antibodies were found by ELISA method in the type III cryoprecipitates.

AIDS-Related Complex↗

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Cryoglobulinemia and liver involvement.

Liver involvement during essential mixed cryoglobulinemia (EMC) and cryoglobulins secondary to acute and chronic liver diseases has often been reported. Cryoglobulins in the serum seem to depend on an abnormal balance between their production and clearance. In this paper, 58 cases of mixed, mainly polyclonal cryoglobulinemia secondary to chronic liver diseases, and 23 cases of EMC, mainly monoclonal, are described. Amongst the EMC, 15 cases have been histologically followed-up and we have shown different types of related pathological liver involvement: from 'pathosis torbida' and chronic persistent hepatitis to chronic active hepatitis and liver cirrhosis. Only a few hepatic lymphoid infiltrates were nodular. According to our data and to those in the literature, we can suggest the following correlations between mixed cryoglobulinemic states and liver diseases: transient polyclonal cryoglobulins are secondary to acute viral liver diseases; polyclonal cryoglobulins are related to chronic liver diseases, initially produced by a polyclonal stimulation; mainly monoclonal, but also polyclonal cryoglobulins in which there is not always liver damage, but nodular infiltrates can be seen at the hepatic site; monoclonal, but also polyclonal cryoglobulins, in which there are nodular lymphoid infiltrates and vasculitis, as in immune complex diseases.

Adult↗

PubMedDate not supplied

Heart involvement in essential mixed cryoglobulinemia.

Cardiovascular involvement has been investigated in the course of essential mixed cryoglobulinemia (EMC). A direct pathogenetic role of cryoglobulins, due to coexisting coronary risk factors, is difficult to demonstrate. Nevertheless, a clear role of cryoglobulins in determining heart damage was hypothesized. A significant statistical incidence (p less than 0.05) of heart impairment was found in patients affected by EMC compared with an age- and sex-matched control group. Cryoglobulinemic coronary vasculitis found at post-mortem examination further supports this point of view.

Coronary Disease↗

PubMedDate not supplied

Chronic-relapsing polyneuropathy in the course of cryoglobulinemia. Clinical aspects and plasmapheretic treatment.

During the last 3 years we have observed 7 cases (4.1%) of cryoglobulinemia-associated polyneuropathy out of 167 patients with demonstrated cryoglobulinemia. Nerve involvement in the course of cryoglobulinemia is usually symmetrical, affecting the distal portions of the limbs and has a chronic or chronic-relapsing evolution. In our experience, the combined treatment by plasmapheresis and glucocorticoids and/or cytostatic drugs has proved to be quite effective, particularly when instituted during the symptomatic exacerbations. Therefore, we suggest to perform a systematic investigation of the cryoglobulinemic phenomenon in every patient presenting with symptoms and/or signs of an apparently idiopathic polyneuropathy.

Adult↗