Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Transition from polyclonal to monoclonal gammopathy resulted in myeloma in the course of cirrhosis is rare but of interest. We treated such a case of multiple myeloma of IgG-kappa type associated with alcoholic cirrhosis. The case was a 72-year-old Japanese male patient who was admitted because of ascites and edema. Physical examination and laboratory findings including liver histology were compatible with alcoholic cirrhosis. Serum electrophoresis revealed monoclonal hypergammaglobulinemia of IgG-kappa. Bence Jones protein in urine was positive. Bone scintigraphy and roentgenography revealed small punched out lesions in the skull. A bone marrow clot section showed marked infiltration of atypical plasma cells. From these findings multiple myeloma associated with alcoholic cirrhosis was diagnosed. On the basis of a review of the reported cases, the possible relationship between monoclonal gammopathy and chronic liver diseases was discussed.
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PMID:IgG-kappa type multiple myeloma in alcoholic cirrhosis--a case report. 190 87

The case of a patient with blue histiocyte syndrome associated to liver cirrhosis and benign monoclonal gammopathy is presented, this having been rarely described in medical literature. We analyse the findings and possible relationship.
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PMID:[Sea-blue histiocyte syndrome, liver cirrhosis and monoclonal gammopathy. Description of a case]. 249 23

An unusual thrombin inhibitor is described in a patient suffering from liver cirrhosis, associated with a polyclonal gammopathy. In all screening tests that are based on thrombin action, the plasma clotting time was prolonged; in contrast, a test using the thrombin-like enzyme thrombin coagulase was not influenced. A specific thrombin inhibitor, present in the patient's plasma, was assumed. Fractionation experiments showed that the inhibitor was associated with the patient's IgG fraction. The IgG fraction dose-dependently inhibited the action of thrombin on fibrinogen and the inhibition of thrombin by antithrombin III, but the amidolytic activity of thrombin was only little affected.
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PMID:Acquired thrombin inhibitor in a patient with liver cirrhosis. 407 47

Polyclonal gammopathy with beta-globulin-gamma-globulin (beta-gamma) bridging has been classically, though not exclusively, described with cirrhosis. We studied two unusual cases that exhibited polyclonal gammopathy with beta-gamma bridging. In the first case, the coexistence of Kaposi's sarcoma appeared with angioimmunoblastic lymphadenopathy. In the second, liver disease developed as a complication of alpha 1-antitrypsin deficiency and retroperitoneal malignant fibrous histiocytoma involving the porta hepatis.
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PMID:Polyclonal gammopathy with beta-globulin-gamma-globulin bridging. Two unusual cases. 616 96

Eleven cases of monoclonal gammopathy associated with chronic hepato-biliary diseases are reported. The gammopathy was benign and the majority of hepato-biliary diseases consisted of cirrhosis complicated or not by hepatocellular carcinoma. The small number of cases reported and the absence of distinctive clinical, biochemical and evolutive features of the gammopathy and hepato-biliary disease suggest that the association was fortuitous.
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PMID:[Relation between monoclonal gammopathy and hepatobiliary disease]. 629 1

The possibility of open tubular capillary electrophoresis for clinical diagnostic use is examined. Capillary electrophoresis was performed in an untreated 50 microns (i.d.) x 100 cm (65 cm to detector) capillary with detection of absorbance at 200 nm. Conditions for the separation of serum proteins without adsorption to the capillary surface were established. Quantitative analyses of serum samples from 38 patients with liver cirrhosis, nephrotic syndrome, or polyclonal gammopathy by capillary electrophoresis were done and the results were compared with those by conventional agarose gel electrophoresis and sodium dodecyl sulfate-polyacrylamide gel electrophoresis. All samples were analyzed in duplicate. We evaluated linearity of response, within-run CV, and the correlation between capillary electrophoresis and agarose gel electrophoresis.
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PMID:Quantitative analysis of serum proteins separated by capillary electrophoresis. 847 68

In the peripheral blood (PB) we detected so-called early plasma cells that might already be committed to entering the bone marrow (BM). By two-colour staining with FITC-anti-CD38 antibody, their intensity (CD38++) of expression of CD38 antigen was between that of germinal centre (GC) B cells (low expression (CD38+)) and that of BM plasma cells (high expression (CD38++)), and their phenotype was CD38++ CD19+ CD10- CD20- CD21+ CD24- CD39+ CD5- VLA-4+ VLA-5- MPC-1- without expression of surface membrane IgM (SmIgM). Morphological and immunological examination of the sorted cells confirmed that they were plasmacytoid cells with expression of cytoplasmic IgG (cIgG). Variations of these early plasma cells were examined in various diseases. In active systemic lupus erythematosus, bacterial septicaemia and liver cirrhosis, early plasma cell levels were significantly increased in PB, and after subsidence of such inflammation (inactive states) these cells returned to normal levels. In contrast, normal early plasma cells were significantly suppressed in myelomas, whilst normal or slightly increased numbers of early plasma cells was found in benign monoclonal gammopathy (BMG). In addition, the number of normal early plasma cells returned to a normal level in myeloma cases with complete responses. Therefore, early plasma cells were identified phenotypically, and an increase and decrease in these cells in PB may reflect mobilization and suppression, respectively, of activated B cells into BM plasma cells.
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PMID:Identification of early plasma cells in peripheral blood and their clinical significance. 856 94

A sixty-one-year-old woman, with cirrhosis, presented with a monoclonal gammopathy of uncertain significance (MGSU). Often in a condition of cirrhosis is present a benign M component hypergammaglobulinemia. The electrophoresis and the immunophoresis showed a dense papraprotein in the gamma-region, an IgG with K light chain, an uncertain Bence-Jones proteinuria, a medullary plasmacytosis (9%), and a following growth of paraprotein were present. Lymphoblastic plasma cell were absent. Treatment with beta-IFN 6 MU for a period of six months and 3 MU for a further period of three months proved ineffective for hepatic disease, but produced a quantitative reduction in gamma-G globulin, the Bence-Jones proteinuria was absent, a reduction in M component and in medullary plasmacytosis. Electrophoresis showed a polyclonal evolution of the gammopathy. Suspension of treatment was followed by de novo rise of monoclonal immunoglobulin. The authors report the use of beta-IFN in the therapy of multiple myeloma.
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PMID:Case report of a monoclonal gammopathy in a patient with chronic hepatitis: effects of beta-IFN treatment. 900 83

Monitoring of posttransplantation lymphoproliferative disorder (LPD) is usually based on imaging, which lacks sensitivity. A prospective study in 911 consecutive recipients of liver transplants was conducted to assess the value of gammopathy monitoring by serum protein electrophoresis (SPE) and to compare it with conventional follow-up methods. Patients systematically underwent SPE testing just before transplantation, at least twice during the first year after transplantation, and once a year thereafter. Patients with LPD underwent SPE testing every month. Immunofixation was done if abnormalities were detected by SPE. Gammopathy was observed in 114 patients, 18 of whom had onset of LPD. In 3 other patients, LPD developed, but no gammopathy was detected before onset of LPD or while LPD was present. Multivariate analyses showed gammopathy (relative risk [RR], 65.3), more than one transplantation (RR, 7.5), and viral cirrhosis (RR, 2.8) to be independent prognostic factors associated with occurrence of LPD. LPD was treated by reducing immunosuppression, with or without chemotherapy, administration of anti-CD20 monoclonal antibody, or surgery. The mortality rate was 24% (5 of 21 patients). Remission, which occurred in 13 patients, was associated with disappearance of gammopathy in 10 patients. In 5 patients, normalization of SPE results preceded the diagnosis of remission based on imaging, by a mean of 4 months. For diagnosis of LPD remission, the positive and negative predictive values of disappearance of gammopathy were 91% and 100%, respectively; and gammopathy monitoring was more sensitive than imaging (100% and 38%, respectively). Gammopathy monitoring is an inexpensive, noninvasive, sensitive way to detect LPD and assess the efficacy of treatment. It could be used routinely in follow-up of recipients of transplants.
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PMID:Detection of gammopathy by serum protein electrophoresis for predicting and managing therapy of lymphoproliferative disorder in 911 recipients of liver transplants. 1152 Jul 79

Early erroneous diagnosis of rheumatic disease is common in subjects with arthropathy due to hereditary hemochromatosis. A 71-year-old male with chronic obstructive pulmonary disease and monoclonal gammopathy underwent hip replacement and was referred to our Department because of altered liver function tests. Test results were negative for hepatitis B surface antigen and hepatitis C virus, and positive for rheumatoid factor. A diagnosis of rheumatoid arthritis had been made on the basis of compatible joint involvement and laboratory data and steroid treatment prescribed. Since his serum ferritin was 3249 ng/mL, genetic testing for hereditary hemochromatosis was carried out and revealed homozygosity for Cys282Tyr mutation in the HFE gene. Liver biopsy disclosed cirrhosis compatible with hemochromatosis. Following a review of the patients' radiographs, the diagnosis of hemochromatosis arthropathy was made. Phlebotomies and family screening for hereditary hemochromatosis were done. The most logical explanation for the positive rheumatoid factor result in this subject are his age and the presence of two chronic diseases involving long-standing antigenic stimulation and monoclonal gammopathy of uncertain significance. It is important to distinguish rheumatoid arthritis from hemochromatosis arthropathy for several reasons: patients with hereditary hemochromatosis do not require corticosteroid treatment; in case of erroneous diagnosis of rheumatoid arthritis, phlebotomy is not started early, and familial genetic counseling is not considered. In male subjects with positive rheumatoid factor and joint and liver disease, hereditary hemochromatosis should be considered. More liberal use of genetic testing is justified in such cases.
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PMID:Hereditary hemochromatosis masquerading as rheumatoid arthritis. 1168 50


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