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Biomedical subjects

E Kaiserling

Publications and source records attributed to E Kaiserling.

At least 181 records · Page 10Linked to original sources

Brown bowel syndrome with manifestation in the gastrointestinal tract and thyroid gland.

Brown bowel syndrome (BBS) is a rare disease accompanied by deposits of lipofuscin predominantly in smooth muscle cells of the gastrointestinal tract. To determine whether cells other than smooth muscle cells show pigment deposition we studied biopsies of the stomach, small and large intestine, and thyroid gland of a 52-year-old male with malabsorption syndrome. Light and electron microscopy found lipofuscin pigment in vascular endothelial cells and in smooth muscle cells of the muscularis mucosae, arterioles and venules of the gastrointestinal tract and thyroid gland. All other cells of bowel and thyroid gland, such as epithelial cells, Schwann cells, nerve fibers, fibroblasts, macrophages and leukocytes, showed no such pigment inclusions. Intracellular lipofuscin deposition is thought to be caused by a deficiency of vitamin E. Such a deficiency existed in our patient and was attributed to a six year history of malabsorption syndrome with complete atrophy of the villi of the intestinal mucosa. Lipofuscin pigmentation has been reported in numerous organs of patients with BBS but not, until now, in the thyroid gland or in endothelial cells of lymph vessels. The origin of lipofuscin pigment is not known exactly. The prevailing opinion is that it derives from degenerating mitochondria. Our findings, however, suggest that autophagocytotic processes may also play a role in the formation of lipofuscin.

Digestive System↗

Hemophagocytic syndrome. Differential diagnostic aspects in a case of well-differentiated malignant histiocytosis.

Diagnosis of malignant histiocytosis (MH) often resembles the solving of an intricate puzzle consisting of clinical symptoms such as lymphadenopathy, splenomegaly, fatigue, fever, and rapid progression, and hematopathological findings such as the presence of atypical histiocytes, especially in blood and bone marrow smears. The lack of one or more of these criteria may greatly impede diagnosis, as in the case of a 45-year-old male with an unusual hematopathological manifestation of MH. The major clinical findings included panhemocytopenia, splenomegaly, and signs of liver dysfunction with severe jaundice. During life, a definite diagnosis could not be established. Histological and cytological evaluation of the spleen following splenectomy revealed a marked increase in histiocytes/macrophages with pronounced hemophagocytosis. These findings were interpreted as a (benign) hemophagocytic syndrome, possibly related to a viral infection. Extensive serological investigations, however, furnished no evidence of a so-called virus- or infection-associated hemophagocytic syndrome. The patient died 5 months after the onset of disease with symptoms of progressive liver failure. Meticulous histological examination of bone marrow revealed a few patchy tumorous infiltrates consisting of dense pleomorphic histiocytes. Thus, a diagnosis of MH was established. This case of MH was unusual with particular regard to its pronounced hemophagocytosis, slight cytological atypia of the histiocytes, and absence of infiltration of lymph nodes.

Blood Cells↗

Immunoreactivity of normal and neoplastic human tissue mast cells.

Immunoreactivity of human tissue mast cells (TMCs) was studied in one case of solitary mastocytoma of the skin, three cases of malignant mastocytosis, and in six lymph nodes with reactive intrasinusoidal increase of TMCs. Immunohistochemically, TMCs reacted positively to antisera against vimentin, common leukocyte antigen (CLA), lysozyme, alpha 1-antitrypsin (alpha 1-AT), and alpha 1-antichymotrypsin (alpha 1-ACT) and to a monoclonal antibody (KiB3) that detects preferentially B-lymphocytes. Additionally, strong positive reactions to polyclonal antisera against adrenocorticotropic hormone (ACTH) and human peptide histidine isoleucine (PHI) and weaker reactions to antisera against leu-enkephalin and met-enkephalin were observed; all other antisera tested yielded negative results. Positive stainings for vimentin, CLA, alpha 1-AT, alpha 1-ACT, and lysozyme further support the hypothesis that human TMCs may be related to the myeloid-monocytic system. The positive reactivity of TMCs to antisera against ACTH, PHI, leu-enkephalin, and met-enkephalin has not been reported previously. These findings suggest that TMCs are able to store and/or produce regulatory peptides in addition to many other well-known, granule-bound mediators.

Humans↗

Lymphoid cells and tissue mast cells of bone marrow lesions in systemic mastocytosis: a histological and immunohistological study.

Systemic mastocytosis (SM) can be regarded as a tumorous proliferation of tissue mast cells (TMC) involving various organs, particularly the bone marrow. The infiltrates, however, are by no means composed exclusively of TMC, but also contain eosinophils and lymphocytes. The varying composition of the TMC infiltrates and the immunohistological characteristics of the lymphatic cells were the main subjects of investigation in this study. Three different types of bone marrow infiltrates could be identified: (1) A pure mastocytic infiltrate. (2) A mixed mastocytic/lymphocytic infiltrate. (3) A predominantly lymphocytic infiltrate containing loosely-scattered TMC. The mixed mastocytic/lymphocytic infiltrate seems to be a unique finding confined to the bone marrow in cases of SM, and is not detected in this conformation in other tissue sites normally involved in SM (spleen, liver, lymph nodes and skin), nor in cases of malignant mastocytosis. The lymphoid cells could be identified immunohistologically as being an admixture of T lymphocytes and B lymphocytes, while NK cells were virtually absent from the composite nodules. The TMC reacted strongly with antibodies (monoclonal or polyclonal) against vimentin, common leucocyte antigen, lysozyme, alpha 1-antichymotrypsin and alpha 1-antitrypsin, but were negative with a variety of other antibodies tested (UCHL1, MB1, Ki-B3, Leu-7, KL1, desmin, S-100 protein, F VIII-related antigen and chromogranin A).

Adult↗

[Progressive lymphangiokeratoma and angiosarcoma (Stewart-Treves syndrome) in congenital lymphedema].

Three unusual clinical and histopathological changes were found in a 59-year-old male patient with congenital lymphedema of the lower left leg. Firstly, a keratotic lymphangioma had developed in the course of only a few years and in the end involved the entire left leg up to the buttocks--far beyond the edematous area. Secondly, concealed beneath the lymphangiokeratoma, a solitary tumor almost the size of a table-tennis ball was found on the outer side of the lower left leg. Histopathologically, the tumor proved to be a malignant angiosarcoma, which had grown over a period of some months. It was characterized histopathologically by solid and angiomatous differentiation. Thirdly, the superficial and deep lymph vessels revealed remarkable atypia and papillary proliferations of endothelial cells. After amputation of the left leg at the thigh, with complete removal of the angiosarcoma, but leaving the pathologically altered vessels in the upper part of the left leg and the buttocks, no clinical signs of metastases of progression have been noted during the first year after operation.

Amputation, Surgical↗

T-cell lymphomas of the stomach: morphological and immunological studies characterizing two cases of T-cell lymphoma.

Using cytochemical, electron microscopic and immunohistochemical techniques in 20 primary malignant lymphomas of the stomach, we found 18 B-cell and 2 T-cell lymphomas. Primary T-cell lymphoma in the stomach has not been previously reported. The T cells in both cases were reminiscent of T immunoblasts with prominent nucleoli and a basophilic cytoplasm. Case 1 showed a cytological relationship to pleomorphic T-cell lymphoma, large cell type. Case 2 contained in addition some cells not previously described in T-cell lymphomas, resembling immature plasma cells with abundant rough endoplasmic reticulum. Focal positivity to acid phosphatase and dipeptidylaminopeptidase IV suggests the T-cell nature of both lymphomas. In both cases the tumour cells were OKT 11 and OKT 4 positive, and negative for OKT 8. Thus, both cases represent high-grade malignant T-cell lymphomas which correspond phenotypically to T-helper cell lymphoma. Case 2 revealed a further immunohistochemical peculiarity: atypical immunoblasts reacted positively with Ki-1 antibody. Thus, it is a Ki-1 lymphoma of T-cell type.

Adolescent↗

Solitary mastocytoma of the eyelid. A case report with special reference to the immunocytology of human tissue mast cells, and a review of the literature.

Solitary mastocytoma (mast cell naevus) of the skin represents a relatively rare dermal tumour. Its occurrence on the lower eyelid is exceptional. We report the case of a 4 month old male infant who exhibited a firm, yellowish nodule (1 cm in maximum diameter) on the lower lid of the right eye from birth. Histologically, the tumour consisted of strongly metachromatic tissue mast cells (TMC) infiltrating the whole dermis, the adjacent subcutaneous tissue and the lid muscle. Since comparable skin lesions in other sites were not observed, a diagnosis of solitary mastocytoma was made. Immunocytological investigations revealed strong reactivity of the TMC to antisera against vimentin, common leucocyte antigen (CLA), alpha 1-antitrypsin (alpha 1-AT) and alpha 1-antichymotrypsin (alpha 1-ACT). A minor proportion of the TMC reacted to antisera against lysozyme and KiB3. Surprisingly, the TMC also reacted to antisera against certain regulatory peptides (RP), namely adrenocorticotropic hormone (ACTH), peptide histidine isoleucine (PHI), leu-enkephalin and met-enkephalin. However, absorption controls revealed that the immunostaining for ACTH and the two enkephalins was non-specific. The immunocytological phenotype of TMC suggests a close relationship to the myeloid-monocytic lineage, but a possible relationship between TMC and the diffuse neuroendocrine system needs further investigation.

Antibodies, Monoclonal↗

Skin tumor of T accessory cells (interdigitating reticulum cells) with high content of T lymphocytes.

A case of T-accessory cell tumor of the skin in a 67-year-old man is reported. The limbs, shoulders, and face were affected, but no visceral involvement is evident 6 years after onset. Tumor cells are nonphagocytic mononuclear cells with folded irregular nuclei. Immunologically, cells were positive for S100 protein, HLA-DR, Ki-M1, Leu 3a (CD4), Leu 6 (CD1); that is, they are identical to the phenotype of Langerhans or interdigitating reticulum cells (IDCs). Birbeck granules were absent. The clinical course appears to be less aggressive than that of the reported IDC sarcomas in other anatomical sites. The similarity of our case to some cases of so-called "non-X histiocytosis" of the skin is discussed. It is suspected that the "non-X histiocytosis of the skin" reported in the literature might have included T-accessory cell tumors, especially those of IDC origin. More immunological studies on the histiocytic disorders of the skin are necessary to clarify their cytogenesis.

Aged↗

Distribution of gastrointestinal hormones in the adaptive response after small bowel transplantation.

Enteroendocrine regulatory peptides may play an important role in the adaptation of small bowel mucosa, and it is likely that they act interdependently with neural and luminal stimuli. We assessed their action in rats by morphometric evaluation of enteroendocrine cells after heterotopic accessory small bowel transplantation (SBT), in which the graft is shunted off from the intestinal passage and is entirely deprived of neural connections, and after orthotopic SBT with normal intestinal passage. Sections of the jejunum and the ileum of the graft were immunostained with antibodies to cholecystokinin (CKK), neurotensin (NT) and vasoactive intestinal peptide (VIP). The amount and distribution of positive cells was assessed semiquantitatively. Three weeks after SBT in the heterotopic graft there was a marked decrease in CCK and NT positive cells and VIP positive fibres in the entire organ, compared with the controls. Histological examination revealed that villi and crypts had atrophied. After orthotopic SBT the number of CCK and NT positive cells increased and exceeded normal values by 20-40%. VIP positive fibres did not reach normal amounts. No mucosal atrophy was detected. These findings support the view that the intrinsic neurohormonal system is reestablished in the grafted small bowel and that enteroendocrine regulatory peptides may act as trophic factors that are responsible for adaptation after SBT.

Adaptation, Physiological↗

Unusual crystalline inclusions in a case of AIDS-related complex.

A lymph node of a patient with AIDS-related complex was studied with light and electron microscopic techniques. In numerous plasma cells and follicular center cells amorphous and unusual crystalline structures which probably consist of abnormal immunoglobulin could be found.

AIDS-Related Complex↗

Pulmonary adenocarcinoma of fetal type: alternating differentiation argues in favour of a common endodermal stem cell.

Pulmonary adenocarcinoma exhibit different cell types that ultrastructurally, appear to be related to the different epithelial cell types occurring in the terminal lung lobule. The present paper describes the light-microscopical, ultrastructural and immunohistochemical features of a newly defined type of pulmonary adenocarcinoma that resembles an early stage of lung differentiation. The alternating epithelial differentiation within this tumour speaks in favour of a common endodermal stem cell for the different types of epithelial cells within the lung, including endocrine cells. The relationship of this tumour to pulmonary blastoma is discussed.

Adenocarcinoma↗

Tumour of Wagner-Meissner touch corpuscles. Wagner-Meissner neurilemmoma.

Two benign tumours composed mainly or exclusively of Wagner-Meissner corpuscles are described. In the first case the touch corpuscles are composed of closely piled laminar cells and surrounded by argyrophilic fibres. In the second case some Schwann cells are observed in between the tactile corpuscles. The light microscopic, electron-microscopic and immunohistochemical results demonstrate that these corpuscles are comparable with the tactile end organs of the skin. Immunohistochemically, neuron-specific enolase, vimentin and protein S-100 could be demonstrated in the tactile corpuscles. Neural processes present in normal Meissner corpuscles are absent and immunohistochemically no nerve fibres or nerve endings can be demonstrated using antibodies to neurofilaments as they are observed in normal touch corpuscles of the skin. Tumours which consist mainly of multiple touch corpuscles have not been described in the literature. It is suggested to call these tumours Wagner-Meissner neurilemmoma.

Adult↗

Mast cell sarcoma of the larynx.

A 74 year old woman presented with a primary subglottic tumour. Neither cutaneous mastocytosis (urticaria pigmentosa) nor spread to the bone marrow, liver, or spleen were detected. About two years after initial manifestation of the tumour nodular skin metastases appeared, as well as local recurrence in the larynx. Despite chemotherapy and radiation the disease progressed and was fatal. The terminal phase was characterised by generalisation of the mast cell tumour with diffuse infiltration of bone marrow and, shortly before death, leukaemic transformation. The patient died four years after onset of disease with symptoms of a hemorrhagic diathesis. As far as we know this is the first case of mast cell sarcoma to be reported in man.

Aged↗

A morphologic study of lymphadenosis benigna cutis.

Two skin biopsies of lymphadenosis benigna cutis have been analyzed by morphological and immunological methods using monoclonal and polyclonal antibodies on cryostat and paraffin sections. Follicular structures containing active germinal centers are composed of identical cell types as germinal centers of normal lymphatic tissue, e.g. centrocytes, centroblasts, immature plasma cells, dendritic reticulum cells and some T lymphocytes. Outside and inbetween the secondary follicles the infiltrate is composed of small T lymphocytes (OKT-3+, focal positive acid phosphatase reaction). Among T lymphocytes the OKT-4+ to OKT-8+ ratio was 2:1. Within these areas, consisting almost exclusively of T lymphocytes, cells with electron microscopical features of indeterminate cells and interdigitating reticulum cells were recognized. Those cells are OKT-6+. It can be concluded that in lymphadenosis benigna cutis the infiltrate of the dermis is composed of B and T cell areas which show the same microarchitecture and morphology as in normal lymphatic tissue.

Antibodies, Monoclonal↗

Immunoelectron microscopic demonstration of tissue antigens with monoclonal antibodies.

Two methods of demonstrating tissue antigens by ultrastructural enzyme immunohistochemistry were tested. The monoclonal antibodies Ki-M1 and Ki-M4 were chosen for testing the methods because Ki-M1 identifies a relatively stable, and Ki-M4 a very unstable antigen. The two antibodies react selectively with human macrophages and interdigitating reticulum cells or dendritic reticulum cells of lymphoid follicles. The Ki-M1 reaction product is confined to the surface membrane. Ki-M4 reactivity is located on the surface membrane and, less often and to a lesser extent, in the cytoplasm. The technical prerequisites for reliable conservation of the antigens identified by these two antibodies were standardized. The results indicated that prior fixation in 4% paraformaldehyde is preferable for optimum preservation of stable antigens. Application of the primary antibody prior to fixation was found to be the best procedure for demonstrating unstable antigens, although nonspecific reactions were seen more often with this method.

Antibodies, Monoclonal↗