Monoclonal immunoglobulin deposition occurs in tissues as Congo Red binding fibrils in light chain amyloidosis, as less structured deposits in light chain deposition disease, and as similar but distinct deposits in light and heavy chain deposition disease. The nonamyloid forms were found in 13 patients who had evidence of plasmacytic dyscrasia by the immunohistochemical detection of immunoglobulin light chains of kappa or lambda class (with or without staining for a single heavy chain isotype) and by the absence of amyloid P component in tissue sections that did not show the birefringence characteristic of amyloid after Congo Red staining. All but two of the patients presented with proteinuria with or without azotemia. Clinical syndromes involving other organ systems were less common but occasionally severe. Four patients had overt multiple myeloma. Three others had hypercalcemia and mild bone marrow plasmacytosis but no lytic lesions. Analyses of immunoglobulin synthesis in bone marrow cells from seven patients showed excess light chains in all and incomplete light chains or heavy chain fragments in six, regardless of whether an intact monoclonal protein or related subunit was in the serum or urine. The fibrillar (amyloidotic) and nonfibrillar forms of monoclonal immunoglobulin deposition occur either in overt multiple myeloma or in the course of less neoplastically aggressive plasmacytic dyscrasias. Bone marrow cells from patients with either type produce immunoglobulin fragments that are related to those deposited in the affected tissues.

Monoclonal Immunoglobulin Deposition Disease: Light Chain and Light and Heavy Chain Deposition Diseases and Their Relation to Light Chain Amyloidosis: Clinical Features, Immunopathology, and Molecular Analysis

Ependym und Circumventriculäre Organe

Light-chain deposition disease: Its relation with AL-type amyloidosis

Chapter 3 The daily light-dark cycle and rhythmic metabolism in the photoreceptor—Pigment epithelial complex

Light chain nephropathy.

To seek a morphological expression of circadian rhythm, we investigated cytologically pineal glands taken from rats every 2 to 4 h under a lighting regime of 12 h of illumination (6:00 to 18:00) and 12h of darkness. The changes in the number of synaptic ribbons and ribbon fields was observed by electron microscopy. The number of these intracellular elements was greatest at 2:00 and lowest at 14:00, the difference being statistically significant. There were no significant local differences in numbers with respect to the part of the pineal gland examined. The data are similar to those of Vollrath from the pineal gland of a guinea pig, and seem to confirm a circadian function in the pineal gland in mammals.

https://link.springer.com/content/pdf/10.1007/BF00219839.pdf

A morphological study of the circadian cycle of the pineal gland of the rat.

To study the effects of bilateral ophthalmectomy on the circadian rhythm of the pineal gland, the number of the synaptic ribbons and ribbon fields in the pineal gland of female rats were determined by electron microscopy. In a preliminary experiment, the pineal gland was taken from rats at 15 days, and 1, 2, 3, 4, and 6 months after bilateral ophthalmectomy. It was found that the numbers of both synaptic ribbons and ribbon fields decreased markedly and reached a minimum at 1 month postoperatively, but recovered completely after 4 months. In the main experiment, the number of these intracellular elements was counted in both control and enucleated animals killed at 4 h intervals between 02:00 h and 22:00 h at either 1 month or 6 months after operation. All animals were kept under a lighting regimen of 12 h of illumination (06:00 to 18:00) and 12h of darkness (18:00 to 06:00). After 1 month, a remarkable decrease in the number of both the synaptic ribbons and ribbon fields was again noted but the circadian rhythm still remained. Complete quantitative and qualitative recovery in the circadian rhythm was obtained 6 months later.

Pineal synaptic ribbons in blinded rats.

An electron microscopic study of a pineal gland which had been obtained from, a 3-year-old girl at autopsy revealed the presence of synaptic ribbon in it. Namely, in some parenchymal cells observed, synaptic ribbons, each of which was ca. 700 nm in length and with synaptic vesicles of up to 60 nm in diameter gathering on the surface, were found in the cytoplasmic area adjacent to the cell membrane. The substructure of the synaptic ribbon, i.e., parallel striae running inside along the longitudinal direction, was definitely observed. There is a hypothesis which has been almost accepted that the mammalian pinealocyte is homologous to the photoreceptor cell of the pineal organ in lower animals, and one of the morphological evidences which supports this speculation is the presence of sensory cell elements in both. Since, this kind of work has been hardly done with human materials, the above finding may offer a meaningful information which discloses a part of the real function of the pineal gland in man.

Synaptic ribbon in the human pinealocyte.

An autopsy case of multiple myeloma (IgG, lambda type), clinically characterized by decreased glomerular filtration rate, is reported with particular emphasis on changes in the glomeruli of kidneys. Histologically, the glomeruli revealed slight increase in mesangial matrix and focal thickening of tuft capillary wall. Electron-microscopically, deposits were observed in a subendothelial location in the glomerular capillary walls, and inclusions were noted in the cytoplasm of the visceral epithelial cells. Histoimmunofluorescent study of the kidney demonstrated intense focal and slight diffuse positivity against labelled antisera of anti-IgG and anti-lambda type of light chain on the capillary wall of the glomerular tufts. Other immunoglobulins were not demonstrable in capillary walls. These findings represent the intraglomerular deposition of paraprotein of multiple myeloma without amyloidosis.

Katsumaro Kurumado

Papers

A morphological study of the circadian cycle of the pineal gland of the rat.

Pineal synaptic ribbons in blinded rats.

Synaptic ribbon in the human pinealocyte.

Glomerular lesions in multiple myeloma.