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Biomedical subjects

F Danon

Publications and source records attributed to F Danon.

At least 55 records · Page 3Linked to original sources

Necrobiotic xanthogranuloma with myeloma. A case report.

Necrobiotic xanthogranuloma is a newly recognized cutaneous manifestation associated with paraproteinemia. Necrobiotic xanthogranuloma is distinctive clinically by yellowish-red nodular lesions, often ulcerated, with predilection for the periorbital area and ocular involvement. Histologically, it is characterized by the combination of inflammatory xanthogranulomas together with areas of necrobiosis. These skin lesions are associated with a dysglobulinemia and preceded the development of an IgG lambda myeloma in a previous patient. Hyperlipidemia may be found in some patients. A case of necrobiotic xanthogranuloma is reported, occurring in a 46-year-old man and associated with an IgG kappa myeloma.

Granuloma↗

[A variety of human autoantibodies recognizes in HeLa cells 2 proteins related to the TFIIIA factor of Xenopus laevis which regularizes the transcription of ribosomal 5S RNA].

Using the sera from two patients with autoimmune disorders, we have identified by immunoprecipitation of HeLa cell extracts two proteins with apparent molecular masses of 37 kDa (p 37) and 32 kDa (p 32). These proteins are associated with 5 S RNA. They are antigenetically related to Xenopus laevis 5 S RNA transcription factor TFIIIA, which is very abundant in early oocytes of this species. In contrast to what is observed in X. laevis oocytes, the TFIIIA-related proteins of HeLa cells are present in very small amounts. Our data suggest that proteins p 37 and p 32 are involved in the control of 5 S RNA transcription.

Animals↗

Waldenström's macroglobulinaemia in a bird breeder: a case history with pulmonary involvement and antibody activity of the monoclonal IgM to canary's droppings.

A canary breeder with Waldenström's macroglobulinemia developed a pulmonary disease related to an interstitial infiltration by malignant B cells secreting a monoclonal IgM K. The serum monoclonal IgM was shown to possess an antibody activity to some canary's droppings antigen. This finding may help elucidate the natural course of lymphoid malignancies and the pathogenesis of rare visceral localizations.

Adult↗

Autoantibodies to nuclear lamin B in a patient with thrombopenia.

We report the characterization of novel nucleus specific autoantibodies in the serum of a patient with systemic lupus erythematosus. Immunofluorescent staining of cycling cells and absorption experiments localized the antigen to the nuclear envelope. Two-dimensional gel electrophoretic analysis of immunoprecipitated nuclear proteins show the antigen to be an acidic polypeptide (IP approximately 5.4) of 68 kDa molecular mass. It has been identified as lamin B, one of the three major nuclear envelope polypeptides of mammalian cells. Antibodies shown to be polyclonal immunoglobulin Gs, were directed against determinant(s) of the protein that have apparently been conserved during evolution. They do not appear to be related to other autoantibodies present in the serum (anti-DNA and anti-platelet). The nuclear specificity shown by these antibodies further demonstrates the antigenicity of proteins related to intermediate filament proteins in patients with autoimmune disorders.

Adult↗

Sicca complex and infection with human immunodeficiency virus.

Five male patients with the persistent generalized lymphadenopathy syndrome also had a sicca complex. Salivary gland biopsy specimens showed diffuse lymphocytic infiltration of the glandular parenchyma. Serum autoantibodies and rheumatoid factor were not detected. All patients had IgG antibodies to human immunodeficiency virus and IgG to the viral capsid antigen of Epstein-Barr virus. These five patients had benign lymphocytic infiltrates in other organs (lung, liver, and kidneys). Sicca complex may be one of the various manifestations of the lymphoid hyperplasia noted in human immunodeficiency virus-infected patients. In these patients, the sicca complex showed specific features related to male predominance, lack of serum autoantibodies, and peripheral-blood T-lymphocyte subset distribution.

AIDS-Related Complex↗

Impaired anti-pneumococcal antibody response in patients with AIDS-related persistent generalized lymphadenopathy.

Pre- and post-immunization serum antibodies to pneumococcal polysaccharides (PPS) and tetanus toxoid (TT) were measured in 25 patients with persistent generalized lymphadenopathy and serum antibodies to the human immunodeficiency virus (HIV). The increase in post-immunization anti-PPS antibodies was lower than 40% in 16/25 patients. Isotype analysis indicated that the IgM, IgA, IgG2, but not the IgG1 antibody responses were lower in patients that in healthy controls, whereas pre-immunization values were similar. For TT, no difference was found between the patients and the healthy group in total and IgG1 antibody response whereas IgG4 response was lower in patients. No significant association was found between the defect in anti-PPS antibody response and associated thrush or constitutional symptoms or other immunological parameters. These findings suggest that defective response to a thymo-independent polysaccharide antigen is a distinctive consequence of HIV infection.

AIDS-Related Complex↗

Spontaneous recurrent fetal wastage and autoimmune abnormalities: a study of fourteen cases.

Case records of 14 women with spontaneous recurrent fetal wastage were analyzed. Venous and/or arterial thrombosis developed in nine patients and vasospastic symptoms in eight. Every patient had some kind of autoantibodies: prothrombin activation inhibitor was present in seven cases, anticardiolipin in five, and, antimitochondrial antibodies in two. Antinuclear antibodies were present in seven cases, and significant titer of antibodies to single-stranded DNA in nine cases. With a mean follow up of 6.5 years no patient developed systemic lupus erythematosus. It is suggested that such women have a peculiar form of vasculitis.

Abortion, Habitual↗

Gastric form of alpha chain disease.

A case of alpha chain disease, involving the stomach only, is reported in an Algerian man suffering from epigastric pains. Upper digestive tract fibreoptic endoscopy showed two antral ulcers and an ulcerative gastritis pattern, which promptly disappeared with cimetidine treatment. Antral biopsies at a distance from the ulcers, but not of the ulcer crater itself, disclosed a dense infiltration of antral lamina propria by mature or sometimes atypical plasma cells. On transmural surgical antral biopsy, the infiltrate spread to the superficial part of the submucosa. No other localisation of the disease was found in spite of multiple biopsies obtained by endoscopy, with a peroral capsule and during staging laparotomy. The alpha chain disease protein was absent from serum and urine, but found in the gastric juice and in the cytoplasma of the cellular infiltrate (alpha 1 subclass). A complete clinical, endoscopic, histological and immunological remission was observed after a six months' course of oral tetracycline.

Adult↗

Serum immunoglobulin G subclass dysbalances in the lymphadenopathy syndrome and acquired immune deficiency syndrome.

Serum IgG subclass levels were measured by an indirect competitive immunoenzymatic assay with monoclonal antibodies in 61 adult patients of different geographic origins affected either with the lymphoadenopathy syndrome (LAS, 46 cases) or with the acquired immune deficiency syndrome (AIDS, 15 cases). In spite of considerable variations from patient to patient, IgG-1 and IgG-3 levels were higher than in normal Caucasians, with IgG-1 levels much more elevated in LAS than in AIDS patients. In Caucasians with AIDS or LAS, IgG-4 levels were low. IgG-2 levels tended to be low and correlated negatively with IgG-1 levels. IgG subclass imbalances were especially striking in patients with lymphoid interstitial pneumonitis. The results suggest that the IgG increase predominantly affects the less T-dependent subclasses. The low levels of the more T-dependent isotypes do not appear to play a clear role in the occurrence of pyogenic infections in certain patients.

Acquired Immunodeficiency Syndrome↗

Direct sensitive immunocharacterization of gangliosides on plastic thin-layer plates using peroxidase staining.

A technique for the immunocharacterization of gangliosides on thin-layer plates using a peroxidase-labelled anti-Ig antibody is described. The technique uses plastic plates and does not necessitate any transfer of the antigen onto nitrocellulose for immunodetection. Being extremely sensitive, it permits the characterization of gangliosides which cannot be detected by classical densitometric techniques and allows the detection of gangliosides even in trace amounts in ganglioside mixtures. It appears to be useful for the screening of monoclonal antibodies because of its rapidity. Its specificity was found to be analogous to other immunological techniques such as complement fixation and agglutination.

Antibody Specificity↗

PSL, an S phase-related p55 nuclear antigen, associates transiently with chromatin.

An S phase-related nuclear 55K antigen, also designated PSL, has been characterized in various mammalian cells, using a human serum from a patient with autoimmune disorders (Barque et al., EMBO j 2 (1983) 743). In this report, we show by immunoelectron microscopy that the p55 protein associates in situ with the chromatin of rat hepatocytes. This association is a transient one, as indirect immunofluorescence studies show that PSL does not bind to individualized metaphase chromosomes. Furthermore, immunoprecipitation tests indicate that the majority of PSL is in the non-chromosomal cell fraction. These results suggest that this nuclear antigen is directly involved in the DNA replication process.

Animals↗

Immunologic markers of Burkitt's lymphoma cells.

Lymphocyte markers were studied on fresh cells from 30 patients with Burkitt (L3) leukaemia and cell lines derived from endemic and non-endemic Burkitt's lymphoma (BL) patients. We observed day-to-day variations of lymphocyte marker expression by cultured lines and, occasionally, differences between fresh and cultured cells. In L3 leukaemia, a wide range of phenotypes, including pre-B cell and mature monoclonal IgM + IgD positive B-cell phenotypes, was observed. Most often, the cells expressed high-density monoclonal surface IgM without IgD and lacked IgG Fc, complement and Epstein-Barr virus receptors. Blast cells from rare patients featured monoclonal IgG or IgA instead of IgM. Cases with light chains of the lambda type were more frequent than those with kappa chains. Monoclonal immunoglobulins were found in serum or urine from eight of 20 patients studied. These results are compared with data from the literature on endemic and non-endemic BL and discussed with respect to the maturation stage reached by the cells. In the study of both fresh and cultured cells, we demonstrated a correlation between variant chromosomal translocations and light-chain types, the cells from patients with a t(2;8) translocation expressing kappa and those with a t(8;22) expressing lambda chains, with one exception Vimentin expression was absent or weak in most BLs studied (lines or fresh cells) and in cells from patients with Langer-Giedion syndrome, in contrast to most other lymphomas and leukaemias and normal lymphoblastoid cell lines.

Burkitt Lymphoma↗

Characterization of a human monoclonal IgM with antibody activity to dsDNA.

We report here the characterization and fine specificity of a human monoclonal IgM lambda reacting with double stranded DNA. The purified IgM and its Fab fragment bind to DNA in the Farr or Crithidia lucilae assays as well as in an immunoenzymatic test. In the latter case, binding was inhibited by native DNA, by DNA alternative co-polymers and by some homopolymers only, but not by monodeoxyribonucleotides and ribonucleotides. Since cardiolipin and phosphatidic acid were good inhibitors, these data suggest that the monoclonal IgM reacts with the sugar phosphate backbone of deoxyribonucleic acids. Interestingly, the patient was affected with both Waldenström's macroglobulinaemia and pernicious anaemia without any symptoms of systemic lupus erythematosus.

Aged↗

Measurement of serum IgG4 levels by a competitive immunoenzymatic assay with monoclonal antibodies.

A competitive indirect ELISA is described for the measurement of IgG4 levels. It uses a monoclonal anti-subclass and antibody and purified monoclonal IgG4 as standards. This method is sensitive and reproducible and more accurate than hemagglutination inhibition and radial immunodiffusion. Serum IgG4 levels in 173 normal adults were less than 0.01-2.1 mg/ml (mean 0.30 mg/ml) in women and less than 0.01-1.87 mg/ml (mean 0.465 mg/ml) in men.

Adult↗

Cryoprecipitation of an anti-Pr2 monoclonal IgM cold agglutinin in the presence of GM3 ganglioside.

The mechanism of cryoprecipitation of a monoclonal IgM kappa cryoglobulin (Mou) with a cold agglutinin activity of Pr2 specificity has been studied. By immunodiffusion this cryoglobulin reacted (by its Fab' fragment) with micellar GM3, a ganglioside bearing the Pr2 antigenic determinant. In contrast to previous reports that indicated a possible temperature dependent self-association of IgM molecules via an immunological interaction leading to cold precipitation, we could not detect any affinity of this cryoglobulin for IgM when we used passive hemagglutination or an indirect enzyme-linked immunosorbent assay (ELISA). However, a GM3-like ganglioside could be extracted, by drastic methods, from the cryoglobulin studied at 22 degrees C, whereas no GM3 was extracted from two control cryoglobulins. Some minor gangliosides (representing less than 25% of total amount of bound gangliosides) were also extracted from Mou cryoglobulin and these gangliosides were shown to crossreact with GM3, as they specifically bind to Mou cryoglobulin by ELISA. After cryoprecipitation the serum still contained a monoclonal anti-Pr2 IgM kappa. A GM3-like ganglioside could be extracted from this purified IgM, and cryoprecipitability could be induced by the addition of a minute amount of micellar GM3. These results suggest that Mou cryoglobulin circulates as an immune complex and that cryoprecipitation may depend on unique IgM-GM3 (or IgM-GM3 cross-reacting gangliosides) complexes.

Agglutinins↗

[A cytoplasmic antigen related to the cell cycle identified by a human autoimmune serum].

A human serum from a patient with an autoimmune disorder, previously characterized for its ability to specifically recognize a chromatin-associated PSL or p55 antigen, was found also to detect a 30-32 K polypeptide in the cytoplasm of human HeLa cells. This molecule is strictly cytoplasmic, does not cross react with nuclear PSL and is synthesized only during the S phase of the cell cycle.

Antigens↗

Characterization by human autoantibody of a nuclear antigen related to the cell cycle.

Using a serum from a patient with an autoimmune disease, we have recently described a novel 55 000-dalton antigen (p55) in the nucleus of several animal cells including human ones. This antigen, designated PSL, was not related to the previously defined antigens recognized by sera from patients with systemic rheumatic diseases (Sm, n-RNP, SS-B, Scl-70). We have now found that p55 is associated with chromatin structures as it is released from the nucleus of mink cell fibroblasts by saline + DNase treatments. Analysis by sucrose gradient centrifugation of the nuclear material released in these conditions indicated that p55 co-migrated with core histones. Meanwhile, p55 was absent from the residual nuclear matrices (achromatinic nuclei). Localization of p55 in synchronized cells was performed by indirect immunofluorescence and immunoprecipitation. P55 appeared to accumulate in the nucleus during the S phase. Finally, it was not recognized by an anti-SV40 tumor serum that specifically precipitated the protein p53, which has been recently related to cell proliferation. Thus, PSL an p53, although apparently not antigenically related, appear to be implicated in the same step of the cell cycle.

Animals↗