The nature of hyaline (eosinophilic) globules and vascular slits of Kaposi's sarcoma.

Ultrastructural studies of Kaposi's sarcoma (KS) from skin biopsies of 24 patients (eight with acquired immunodeficiency syndrome (AIDS) and 16 without) were performed to delineate the nature of hyaline globules and vascular slits. These structures have been regarded as one of the important criteria for the recognition of KS under light microscopy. Histochemical and immunochemical studies were also performed to correlate with the electron microscopic (EM) observations. The most remarkable EM findings of KS were the intracytoplasmic lumen formation and erythrophagocytic activities of the neoplastic cells, particularly in the mature nodular, or neoplastic stage. The spindle-shaped or ovoid neoplastic cells frequently contained one to several intact and fragmented red blood cells. The intracellular and extravasated erythrocytes were often arranged in single files, giving these vascular slits an elongated appearance on longitudinal sections. The phagocytic activities of the neoplastic cells were demonstrated by the presence of membrane-bound lysosomes containing phagocytized erythrocytes and their partially digested forms (erythrophagosomes) adjacent to pinocytotic vesicles, prominent rough endoplasmic reticulum, and Golgi apparatus, as well as scattered, small, membrane-bound lysosomal granules, some of which were attached to the erythrophagosomes. The erythrophagosomes underwent various stages of disintegration. The partially digested red cells varied from 0.4 to 10 microns in diameter. The results of histochemical and immunochemical findings also strongly suggested that erythrophagosomes were most likely the hyaline globules (bodies) seen in light microscopy. The exact mechanism of erythrophagocytosis is uncertain. However, its consequences, erythrophagosomes, and intracytoplasmic lumen formation, particularly in the nodular or neoplastic stage in patients with and without AIDS, are among the important histologic features of KS.

Immunolocalization of basement membrane molecules in the stroma of salivary gland pleomorphic adenoma.

In order to determine the participation of basement membrane molecules in formation of its characteristic stroma, 30 cases of salivary gland pleomorphic adenoma were examined by immunohistochemical staining for type IV collagen, laminin, heparan sulfate proteoglycan, and entactin. The stroma was histopathologically classified into four types: hyaline, fibrous, myxoid, and chondroid. Immunohistochemically, type IV collagen and laminin were more intensively localized in hyaline, fibrous and chondroid types of stroma, whereas heparan sulfate proteoglycan was more prominent in myxoid areas. The results suggest that the stroma contains these basement membrane components, which are possibly biosynthesized by epithelial tumor cells, and that histological variety of the stroma depends on proportion of local contents of each basement membrane molecule.

Neuronal intranuclear hyaline inclusion disease: report of a case and review of the literature.

A case of neuronal intranuclear hyaline inclusion disease (NIHID) is described. The patient was a 26-year-old man who died of a progressive neurologic disorder, the onset of which occurred at the age of 11 years. Clinically, the disease presented as juvenile parkinsonism, and pathologically it was characterized by multiple-system degeneration in conjunction with the ubiquitous presence of intranuclear hyaline inclusions in neurons of the central and peripheral nervous system including the autonomic ganglia. Smaller and less eosinophilic intranuclear inclusions were also present in a small number of glial cells. The neuronal inclusions emitted a strong yellow-green autofluorescence under ultraviolet light and were composed of filaments 10-15 nm in diameter. The glial inclusions also consisted of similar filaments but their autofluorescence could not be determined with certainty because of their small size and background autofluorescence. A review of the literature revealed 19 similar autopsy cases up to 1987. Since the clinical presentation and distribution of neuronal loss as well as the characteristics of the inclusions showed some differences among the cases, some authors speculated that NIHID represented more than one variant of a multiple-system degenerative disease. However, about half of the reported cases had favorable sites of neurodegeneration, such as the pallidum, substantia nigra, motor nuclei of the brain stem, anterior horn cells, Clarke's column and spinal ganglion as well as similarities among the inclusions. Thus, there seems to be a discrete group among cases of NIHID.

Peyer's patches in experimental Salmonella dublin infection in calves. Microvascular and epithelial changes contributing to atrophy of lymphoid follicles.

Six calves were infected per os with Salmonella dublin and killed nine hours to seven days later. Early changes included occlusion of capillaries with a hyaline material, particularly in the ileal Peyer's patch (PP). Central areas of the follicles contained hemorrhages and edema. In later stages the follicle-associated epithelium (FAE) of both the jejunal and ileal PP was fused with the adjacent epithelium and the follicles were collapsed. As judged from 5'nucleotidase histochemistry, follicles were depleted of lymphocytes whereas reticular cells were retained. Carbonic anhydrase (CA) histochemistry showed a decreased reaction in the ileal FAE and a reduced amount of CA reactive material in the follicles of the ileal PP, indicating loss of FAE differentiation and function. Hyaline material and fibrinous thrombi were seen occluding the blood capillaries and the lymphatics, respectively. The villi were atrophied and covered with thick fibrin deposits. Using antifibrinogen antibodies, immunoperoxidase stained fibrin in the lymphatics and the lumenal deposits but not the hyaline material in the capillaries. Reaction for CA indicated that this hyaline material originated from erythrocytes. Factors contributing to the follicle atrophy may include anoxia due to stasis in the microcirculation with the formation of erythrocyte thrombi, and reduced lymphopoiesis due to a decrease in the stimulating factors provided by the FAE.

Globular hyaline masses in the stroma of ameloblastoma: histopathologic and histochemical study.

A microscopical study of 15 intraosseous ameloblastomas revealed the presence of globular hyaline masses in only one of them (6.6%). These masses were confined to the stroma of plexiform areas and especially in regions undergoing cystic degeneration. They were periodic acid-Schiff positive and showed an affinity for acid dyes such as phloxine, acid fuchsin, orange G and picric acid. Regarding their pathogenesis, they are probably formed from extravasation of plasma glycoproteins, condensing and taking up a spheroidal shape in the liquid micro-environment of stromal degeneration under the influence of capillary phenomena.

Clinical significance of serum antibodies against alcohol-altered hepatocyte membrane in alcoholic liver disease.

The clinical significance of serum antibodies against alcohol-altered rabbit hepatocytes was evaluated in 91 patients with alcoholic liver disease. Patients were divided into two groups according to the presence or absence of those antibodies at the time of first admission, and their clinical, biochemical, and histological findings were compared. In 38 seropositive patients, the total amount of ethanol consumption as well as serum activity of glutamate-oxaloacetate transaminase, serum levels of total bilirubin, gamma-globulin, IgG, and IgA were all significantly higher than in 53 seronegative patients. In addition, the extent of fibrosis, alcoholic hyalin, cholestasis, and lymphocytic and polymorpholeukocytic infiltrations in the liver were all significantly greater in the seropositive patients. Histological changes were compared in the 16 patients who continued to drink alcohol and who received repeated liver biopsies. Seven out of eight patients who were seropositive at the time of the first liver biopsy showed histological deterioration, whereas one of the eight seronegative patients showed a slight progression. These results suggest that the presence of serum antibodies against alcohol-altered liver cell membrane delineates a group of alcoholic patients with severe, advanced liver disease characterized by a tendency to progress with continued alcohol ingestion.

Hyaline cells in chondroid syringomas. A light-microscopic, immunohistochemical, and ultrastructural study.

The origin and significance of hyaline cells (HC) in chondroid syringomas (CS) is unclear. In a review of 20 CS, we found HC in eight cases. These were studied immunohistochemically, using antibodies to cytokeratin, vimentin, carcinoembryonic antigen, epithelial membrane antigen, desmin, muscle-specific actin, S-100 protein, myoglobin, alpha-1-antitrypsin, chromogranin, glial fibrillary acidic protein, and neuron-specific enolase. HC gave a positive reaction to cytokeratin, vimentin, S-100 protein, and neuron-specific enolase, but not to the other antibodies. Thioflavin-T stain for amyloid was negative. Ultrastructurally, HC contained intermediate filaments in hap-hazard arrangement without specific structures or densities, and had sparse intracytoplasmic organelles and rare desmosomal attachments. Intermediate forms between epithelial cells and HCs were also identified. Our results suggest that HCs are relatively common in Cs (40%) and are more frequent in CS with solid nests and myxoid stroma (relative frequency 62.5%). Immunohistochemically, HCs of CS have a specific phenotype profile; however, ultrastructurally, HCs of CS are not distinct and are similar to HCs in other organs. These results do not suggest an exclusive myoepithelial origin of HCs; it appears that HCs could derive from any epithelial cell type of sweat glands, probably via a regressive process.

Pulmonary adenosquamous carcinomas with amyloid-like stroma.

Two unique cases of pulmonary carcinomas showing squamous and glandular differentiation and the production of excessive amounts of extracellular waxy eosinophilic material resembling amyloid are reported. Immunohistochemical studies showed strong staining of the neoplastic cells with antibodies directed at cytokeratin, epithelial membrane antigen, and carcinoembryonic antigen. Of particular interest was the simultaneous strong staining for S-100 protein and vimentin. The histology, ultrastructure, and immunohistochemical findings suggest focal myoepithelial differentiation in these mixed carcinomas and indicate an analogy to salivary gland neoplasms, particularly adenoid cystic carcinoma and dermal analog tumors.

Histologic and statistical studies on hyalin bodies in the human endolymphatic sac.

Hyalin bodies are amorphous, eosinophilic masses that protrude from the subepithelial connective tissue into the lumen of the endolymphatic sac. In this study, hyalin bodies were analyzed in two groups of temporal bones: normal bones and bones with cochlear otosclerosis. The results revealed that bones with cochlear otosclerosis had significantly more and larger hyalin bodies that did normal bones. In addition, the hyalin bodies in cochlear otosclerosis were denser and associated with more edema and loose connective tissue in the surrounding areas. Foamy macrophages, concentric calcific structures, and bony ingrowth were frequent features of the hyalin bodies in the cochlear otosclerosis. Our current hypothesis is that these hyalin bodies are repository of membranous cellular debris phagocytized by the macrophages. If this is true, the hyalin bodies may further support the proposed resorptive and phagocytic functions of the endolymphatic sac and the enzymatic concept in cochlear otosclerosis.

Electron microscopy of hyalin bodies in the human intraosseous endolymphatic sac.

Hyalin bodies are amorphous, eosinophilic masses protruding from the subepithelial connective tissue into the lumen of the intraosseous endolymphatic sac (ES). We studied hyalin bodies at the electron microscopic level. Celloidin- embedded temporal bone sections known to have hyalin bodies were re-embedded into plastic and cut into thin sections appropriate for electron microscopy. The results revealed that the hyalin bodies are composed predominantly of thick bundles of collagenous fibers arranged in various directions. Fibroblasts and disintegrated macrophages were occasionally observed among the collagen fibers. Concentric calcific structures found within the hyalin bodies were composed of multiple smaller, concentric, lamellar calcifications. The results of this study support the hypothesis that the hyalin bodies are a repository of membranous cellular debris phagocytized by the macrophages.

[Hyalin substance in odontogenic cysts. Histological observations]. / Materiale ialino nelle cisti odontogene. Osservazioni istologiche.

The odontogenic cysts contain the hyaline deposits in 69.2%. The hyaline deposits appear as a zone that was continuous with basament membrane or as an irregular deposit within the connective tissue.

[Molecular heterogeneity of proteoglycan aggregates of human hyalin cartilage in normal conditions and in systemic bone dysplasia]. / Molekuliarnaia geterogennost' komponentov proteoglikanovykh agregatov gialinovogo khriashcha cheloveka v norme i pri sistemnykh kostnykh displaziiakh.

Components of proteoglycan aggregates of human hyalin cartilage were studied under conditions of normal state and in some forms of osteochondrodysplasia. Extraction of uronic acids and protein from the tissue, amount of fractions and electrophoretic mobility of proteoglycan monomers, rations protein/glycosaminoglycans, keratan sulfate/chondroitin sulfate, a level and type of sulfatation as well as molecular mass of chondroitin sulfate, amino acid composition of rod protein, heterogeneity of binding proteins (concerning their isoelectric points and molecular masses) and immunoreactivity of protein moiety in proteoglycan aggregates were studied in rib cartilage, knee joint and ala ossis ilii. Structural parameters of proteoglycan aggregates proved to be dissimilar and depended on cartilage localization and age of the donors. Impairments in the rate of chondroitin sulfate sulfatation were detected in achondrogenesis of the II type and in diastrophic dysplasia; an extraction ability and amount of proteoglycan fractions, relative content of glycosaminoglycans and binding proteins were altered in some other forms of osteochondrodysplasias. Numerous biochemical markers of extracellular matrix deterioration were detected, which are typical for various morphofunctional alterations in hyalin cartilage--hyperproliferative reactions, tissue prematuration, persistence of the embryonal type of metabolism.

Branch retinal vein occlusion associated with optic nerve drusen: a case report.

Proliferative retinopathy secondary to a branch vein occlusion developed in an otherwise healthy 24-year-old women who also had optic nerve drusen. Since the patient sustained a preretinal hemorrhage, quadrantic photocoagulation was applied to the nonperfused quadrant of the retina involved in the occlusion. This case illustrates that compression of a branch retinal vein is another potential complication of optic nerve drusen.

An autopsy case of atypical infantile motor neuron disease with hyaline intraneuronal inclusions.

We describe a 5-year-old boy who had had a progressive motor weakness with bulbar palsy and spasticity of the lower extremities since age 3 years and who died of bronchopneumonia after about two and a half years of the illness. Neuropathologic examination revealed combined degenerative processes in the upper and lower motor neurons, the spinocerebellar and olivocerebellar systems, and the ventral thalamic nuclei. Lewy body-like intraneuronal hyaline inclusions, which ultrastructurally showed irregular accumulations of trilaminar membranous profiles, were detected in the spinal anterior horn, Clarke's dorsal nucleus, facial nerve nucleus, inferior olivary nucleus, and substantia nigra. This case could be considered as a unique form in the group of the infantile motor neuron diseases associated with multi-systemic degenerations in the central nervous system.

Immunocytochemical and ultrastructural study of Lewy body-like hyaline inclusions in familial amyotrophic lateral sclerosis.

Lewy body-like hyaline inclusion (LI) in the neuronal soma and swollen cord-like processes is a characteristic feature in the anterior horn cells and neurons in thoracic nucleus (Clarke) of familial amyotrophic lateral sclerosis (ALS) with posterior column involvement. We have studied the LI in the case of two sisters with this disorders. Microscopically the LI consists of an eosinophilic "core" surrounded by a basophilic "halo". Ultrastructurally the core consists of granule-associated filaments, while the halo consists of normal-looking neurofilament. Immunocytochemistry with anti-ubiquitin antibody shows that these granule-associated filaments in the core are highly ubiquitinated, while the normal-looking neurofilaments in the halo are not recognized by anti-ubiquitin antibody. Our study proves that LI consists of an aggregation of ubiquitinated filaments among a neurofilamentous accumulation, possibly representing a form of neuronal cytoskeletal disorganization in familial ALS.

Juvenile hyaline fibromatosis. A histologic and histochemical study.

Histochemical and routine light microscopic studies were performed in nodular skin lesions excised from one patient with juvenile hyaline fibromatosis. The lesions had different times of evolution. Recent lesions showed a high density of fibroblastlike cells embedded in an amorphous matrix of glycoproteins, hyaluronic acid, and small amounts of chondroitin sulfates A and C and of dermatan sulfate. The progressive enlargement of the lesions was due to an increase in the amount of intercellular matrix produced by the cells that progressively displayed a pattern of peripheral stratification. In the older lesions, the matrix was mainly composed by chondroitin sulfates A and C. We suggest that juvenile hyaline fibromatosis represents a disease of the connective tissue with progressive abnormal differentiation to chondroid tissue.

A calcium-insoluble 6.4 S protein derived from sea urchin cortical granule exudate.

A major protein component of the sea urchin, Strongylocentrotus purpuratus, cortical granule exudate has been purified and characterized. In the absence of divalent cations, the native, soluble protein has a sedimentation coefficient at infinite dilution of 6.4 S and a molecular weight from sedimentation equilibrium measurements of 2.8 +/- 0.3 X 10(5). These and other data indicate that the protein assumes an elongated, rod-like structure in solution. The protein is greater than 95% homogeneous as judged by agarose- and sodium dodecyl sulfate-gel electrophoresis. In the latter experiments, the protein shows a relative molecular weight of 1.8 X 10(5) and is clearly distinct from the 11.6 S protein described earlier which shows two bands corresponding to 3.2 X 10(5) and 2.1 X 10(5). The 6.4 S protein is the major protein of the calcium-insoluble fraction of cortical granule exudate and contributes to the formation of the extracellular investments of the sea urchin embryo. Using a light-scattering assay, we show that the purified protein retains the ability to aggregate in the presence of divalent cations mirroring its assembly in vivo. Calcium ion alone is able to initiate this reaction and the rate of precipitation increases with calcium concentration. Magnesium alone is ineffective in this regard but, in combination, the two ions act synergistically. Strontium and barium can substitute for calcium, but higher concentrations of the former cations are required to produce an equivalent effect.

Adenomas in glycogen storage disease type 1. Two cases with unusual histologic features.

Two cases of hepatic adenomas arising in patients with glycogen storage disease type I are presented. In both cases, the adenomas showed unusual histological features including marked fatty change, a polymorphonuclear infiltrate, Mallory hyalin, and lamellar fibrosis. In addition, one case was associated with amyloidosis. Such features have only recently been described in adenomas and have not been reported previously in association with glycogen storage disease type I.

Hepatic hyaline globules associated with passive congestion.

We describe the presence of hyaline globules in liver associated with hepatic congestion. These globules were present in hepatocytes in 15% of 100 adult autopsies. They are periodic acid-Schiff (PAS)-positive and diastase-resistant by light microscopy, and in one case examined by electron microscopy, contained microfibrillar material with occasional rod-shaped inclusions. The majority of them stained positively for fibrinogen and alpha 1-antitrypsin by immunohistochemical methods. Of possible etiologies, the globules appear most probably as phagosomes containing imbibed serum proteins under conditions of increased sinusoidal pressure with passive congestion. They are more common than generally realized, when compared with other reported forms of nonglycogenic hyaline globules in liver. They must be distinguished from alpha 1-antitrypsin-disease globules.

Differential response to compressive loads of zones of canine hyaline articular cartilage: micromechanical, light and electron microscopic studies.

Thin (100 micron) perpendicular slices of canine femoral condylar cartilage were placed horizontally on the stage of a Nachet microscope and viewed by transmitted light in the differential interference contrast mode. Each slice was held on the microscope stage by a loading rig and tested mechanically in compression. Measured loads to a maximum of approximately 2-3 MN/m2 were applied to the end of the slice corresponding to the articular surface. Photographs were taken of the cartilage before and during loading, and the distance by which selected chondrocytes were displaced was used as an index of mechanical strain, i.e., of change in length/original length. Maximum strains were observed in the superficial cartilage zone. Minimum strains were recorded in the mid-zone, at a depth corresponding to approximately 75% of the total cartilage thickness. The relative concentrations of cartilage collagen (COL) and proteoglycan (PG) were assessed by the light and electron microscopic histochemical study of cartilage sections taken from contiguous blocks. Superficial cartilage, which deformed most, had high concentrations of oriented COL fibres, low concentrations of PG. Mid-zone cartilage, which deformed least, had lower concentrations of randomly arrayed COL fibres but relatively high concentrations of PG.