Ultrastructural lipid and glycoconjugate cytochemistry of membranous lipodystrophy (Nasu-Hakola disease)

Summary In order to assess the lipid and glycoconjugate characteristics of membranous lipodystrophy, a 29-year-old male with this disease was studied using an ultrastructural cytochemical approach. The specific membranocystic lesions of the disease are composed of cystic spaces and the lining membranes. The membranes were observed to have a two-layered character: microtubular structures in the layer adjacent to the spaces and a central amorphous zone. Lipid staining and the lipase digestion test revealed triglycerides localized not only in the cystic spaces but also in the microtubular structures. Lectin histochemical examintion of carbohydrate components demonstrated thatMaclura pomifera agglutinin bound strongly to the membranes, whileGriffonia simplicifolia I,G. simplicifolia II,Concanavalia ensiformis andTriticum vulgaris agglutinin reacted weakly. Our results indicate the presence of triglycerides and carbohydrates with mainly-D-galactose residues in the distinctive membranocystic lesions, in particular in the microtubular structures.     

Relationship between serum interleukin-4 or interferon-gamma level and B cell abnormality in patients with collagen vascular diseases]

The role of interleukin-4 (IL-4) and interferon-gamma (IFN-gamma) in the pathogenesis of the collagen vascular diseases was studied. The serum level of IL-4 was decreased in patients with systemic lupus erythematosus (SLE) and mixed connective tissue disease (MCTD), and was variable in patients with rheumatoid arthritis (RA). The serum level of IFN-gamma was increased in patients with SLE, MCTD and RA. In patients with SLE, there was an inverse correlation between the levels of IL-4 and IFN-gamma. The proliferation and immunoglobulin production of the high-density-B cells in response to IL-4 was suppressed in normal controls, although the degree of suppression was less in patients with SLE. Cell cycle analysis using ethidium bromide demonstrated the similar findings. These data suggest that IL-4 and IFN-gamma might participate in regulating both of growth and differentiation of B cells in vivo. However, immunoglobulin production by whole B cells in response to IL-2 or PHA-induced T-cell factor was extensively facilitated by IFN-gamma in patients with SLE. It is possible that IFN-gamma enhances the differentiation of already-activated B cells, and that polyclonal B cell activation is promoted. Therefore, the failure of the regulatory mechanism by these cytokines might be related to the pathogenesis of these diseases.     

Electric charge and sugar chains of immunoglobulin G in systemic lupus erythematosus

Background The charge condition of anti-DNA antibody is thought to be closely related to the pathology of renal disorders in systemic lupus erythematosus (SLE). Methods We examined the relationships among the electric charge of IgG, proteinuria, and DNA binding capacity in SLE patients. Abnormal sugar chains, which affect the IgG charge, were also studied. Results IgG in SLE patients with proteinuria had a higher positive electric charge than that in SLE patients without proteinuria, healthy individuals, and patients with other collagen diseases. Anti-DNA antibody titers measured by enzyme-linked immunosorbent assay (ELISA) were high in the positive charge regions of the IgG fractions obtained by a cationic exchange column. The binding capacity of double-stranded (ds)DNA measured by the Farr assay tended to be present specifically in the positive charge region in SLE patients with proteinuria. In affinoblotting using lectins, the percentage of IgG deficient in negative-charged sugar chains was found to be high in patients with a preponderant positive charge on anti-DNA antibody. Conclusions Abnormal IgG-bound sugar chains result in a preponderantly positive charge and are involved in the etiology of SLE nephritis.     

A case of adult-onset Still's disease complicated by non-Hodgkin's lymphoma

Left cervical lymphadenopathy developed in a 50-year-old male who had a history of adult-onset Still's disease for the preceding 18 months. Still's disease is characterized by rash, fever, and leukocytosis. Lymphadenopathy has been reported in about 60% of the patients, and most histopathologic studies have shown non-specific reactive hyperplasia. However, in this case, an open biopsy of the cervical node revealed a histology of diffuse large B-cell lymphoma. The B-cell malignant lymphoma that developed may have resulted from a sequential progression of a previous stage of benign lymphoproliferative lesion. Our case suggests that the pathophysiology of adult-onset Still's disease involves the stimulation of lymphoid systems to the point of progression towards lymphoma. Malignant lymphoma should be added to the list of life-threatening complications which, although rare, are associated with this disease.     

Giant cell tumor of the distal femur: Outcome beyond 20 years of follow-up after curettage with polymethylmethacrylate

Background

Polymethymethacrylate (PMMA) is often used to reconstruct defects after curettage of Giant Cell Tumors (GCT). While GCTs usually originate in the epiphysis, the use of PMMA in distal femoral lesions may induce the risk of degenerative osteoarthritis (OA). We investigated the limb function of patients after curettage with PMMA beyond 20 years of follow-up.