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Biomedical subjects

H E Schaefer

Publications and source records attributed to H E Schaefer.

At least 19 recordsLinked to original sources
PubMed1995

Delineation of the dendritic cell lineage by generating large numbers of Birbeck granule-positive Langerhans cells from human peripheral blood progenitor cells in vitro.

It is well established by in vivo and in vitro studies that dendritic cells (DCs) originate from hematopoietic progenitor cells. However, the presumed intermediate of Birbeck granule (BG)+ Langerhans cells (LCs) has not been detected in cultures derived from bone marrow or peripheral blood progenitor cells (PBPCs), thus contrasting with the data obtained with cord blood. We show here that large numbers of BG+ LCs can be generated from human CD34+ PBPCs in vitro, when granulocyte-macrophage colony-stimulating factor and interleukin-4, potent promotors of LC/DC differentiation, are combined with a cocktail of early acting hematopoietic growth factors. LCs were found to emerge from CD33+CD11b+CD14- progenitor cells that they share with the monocytic lineage. During culture, these cells exhibited a sequence of dramatic morphologic changes, starting with a major increase in granularity followed by an increase in size herein exceeding that of all peripheral blood cells. At the same time, CD1a and major histocompatibility complex class II expression were upregulated and virtually all CD1a++ cells were BG+ by electron microscopy. With prolonged culture, CD1a was downregulated on a major population of cells, paralleled by a loss of BG and an increase of CD4, CD25, and CD80 expression that may correspond to the maturation of epidermal LC in vitro. However, these cells were consistently CD5- and did not exhibit changes in the CD45-isoform expression during culture. The availability of large numbers of these highly purified BG+ LCs and mature DCs allows for specific analysis of these subpopulations and provides a source of potent antigen-presenting cells from individual patients for vaccination protocols against infectious or tumor-associated antigens.

Antigen-Presenting Cells↗
PubMed1995

Progressive liver failure in a patient with adult Niemann-Pick disease associated with generalized AL amyloidosis.

We report a case in which an adult form of Niemann-Pick disease (type B of NPD) was associated with a rapidly progressive generalized AL amyloidosis of kappa type. Both diagnosis were made by biopsy, the NPD by bone marrow biopsy and fibroblast culture, the amyloidosis by liver biopsy. Malignant non-Hodgkin lymphoma was not found. The patient, a 67-year-old woman, died from hepatic coma subsequent to a progressive liver failure. We discuss possible relations between the lysosomal storage disease and the development and rapid progression of amyloidosis.

Aged↗
PubMed1995

Penile ossification and acquired penile deviation.

We report on 3 patients with penile deviation during erection caused by ossification in the corpora cavernosa. In each case hard plaques could be palpated. These indurations were removed through a dorsal longitudinal incision. Histologically, solid bone was demonstrable. Two patients were able to resume normal sexual intercourse, but one became impotent following postoperative cavernitis. Penile ossification is rare in man, and its etiology is unknown. It bears no relationship to the os penis normally present in many other mammals. Diagnosis is best made by palpation and X-ray examination. The treatment of choice for symptomatic ossification is surgical excision.

Adult↗
PubMed1995

Translocation t(8;13) in a patient with T cell lymphoma and features of a myeloproliferative syndrome.

A 32-year-old white woman was admitted with a diagnosis of T lymphoblastic lymphoma and a bone marrow and peripheral blood cytology that was suggestive of a myeloproliferative syndrome (MPS). In addition, islets of myeloid precursors were found in the lymph node where the lymphoma had been diagnosed. Cytogenetic examination was negative for the Philadelphia chromosome (Ph) as well as the RT-PCR for bcr/abl rearrangement, but surprisingly a t(8;13)(q10;p10) was detected. To our knowledge, this translocation has not been reported in such a clinical setting. The patient was treated for the lymphoblastic lymphoma and underwent autologous bone marrow transplantation. She has been in complete remission since induction chemotherapy with a Karnofsky score of 100%. The difficulty of classifying this case is discussed.

Adult↗
PubMed1994

[Treatment of clozapine-induced agranulocytosis using granulocyte colony-stimulating factor].

A 20-year-old woman had for the preceding 11 weeks been receiving clozapine (225 mg/d) for an endogenous psychosis when she developed a urinary tract infection with fever. The blood count showed 2100 white cells/microliter without any neutrophils, the count having been normal 5 days previously. Physical examination was normal except for a fever of 39 degrees C and parodontitis. The red cell count was 3.9 mill/microliters, platelet count 443,000/microliters. Bone marrow biopsy revealed almost complete stop of proliferation and maturation in granulocytopoiesis so that granulocyte colony-stimulating factor (300 micrograms daily subcutaneously) had to be administered in addition to supportive measures. The granulocyte count at first fell to 1400 cells/microliter, but nine days after starting the drug myeloblasts, promyelocytes and myelocytes reappeared in peripheral blood for the first time. On the tenth day, administration of the growth factor was discontinued. An overshoot granulocytopoiesis occurred in bone marrow on the 13th day; on the 22nd day after treatment had been started the patient had a normal blood picture and was discharged.

Adult↗
PubMed1994

The expression of lysozyme in multiple myeloma.

Lysozyme, a hitherto myelomonocytic marker, has been previously reported as being raised in the sera of some myeloma patients. This fact, and the sporadical observation of a positive immunohistochemical lysozyme staining seen in some myelomas, prompted us to systematically search for an expression of lysozyme in both neoplastic and reactive plasma cells. A total of 74 paraffin-embedded, formalin-fixed, EDTA-decalcified core biopsies of newly diagnosed cases of plasmacytoma were immunohistochemically investigated for lysozyme expression by a modified avidin-biotin immunoperoxidase technique. The myelomas were subclassified according to their nuclear maturity into poorly differentiated plasmacytoma (PDP) (30 cases), moderately differentiated plasmacytoma (MDP) (24 cases), and well differentiated plasmacytoma (WDP) (20 cases). An unexpected lysozyme positivity was seen in 16/74 cases, and was most prevalent in 10/30 cases of PDP. No correlation has been detected between either lysozyme and kappa or lambda light chain expression, or an abnormal activity of chloroacetate esterase sometimes seen in myeloma. Since lysozyme has not been found in normal plasma cells or reactive plasmacytosis, the expression of this antigen in myeloma represents another example of so-called lineage infidelity, and parallels the previously reported abnormal expression of other myelomonocytic markers in some myelomas and a myeloma cell line. Apart from the unsettled prognostic impact, a facultative lysozyme expression in myeloma must be always considered when applying algorhythmic immunohistological strategies in delineating the histogenesis of haematopoietic or lymphatic malignancies.

Adult↗
PubMed1992

[The solitary pulmonary histoplasmoma].

A 63-year-old man who grows orchids as a hobby, fell ill with weakness and pain in his hips and legs 2 months after his latest trip to South America (Ecuador). The WBC count was 9900/microliters with unremarkable differential count while blood sedimentation rate was raised to 60/100 mm. The chest X-ray demonstrated in the right upper lobe a well-circumscribed coin lesion (3 cm diameter) of soft-tissue density, uncalcified and without cavitation. Computed tomography in addition revealed an enlarged lymph-node at the lower hilar pole, but no mediastinal lymphoma. Bronchoscopy demonstrated narrowing of a subsegmental ostium of the 6th segment on the right. An attempt at transbronchial biopsy failed. As a peripheral bronchial carcinoma was suspected, a posterolateral thoracotomy was performed (4 months after the trip to Ecuador). Rapid histological examination was negative for tumour and the lesion was therefore enucleated. Histologically (Grocott silver staining) a histoplasmoma was diagnosed. Several serum samples were positive for precipitating (M-band) and complement-binding antibodies (titre 12 days preoperatively was 1:16). The postoperative course was without complication. No anti-histoplasma antibodies were demonstrable 1 year postoperatively.

Antibodies, Fungal↗
PubMed1992

Primary thymic carcinoid with Cushing's syndrome.

In a 52-year-old Caucasian man osteopoikilosis had been misdiagnosed roentgenologically 2 years before his death. Gradually he developed Cushing's syndrome and ultimately superior vena caval obstruction. At autopsy a primary thymic carcinoid with extensive osteoblastic bone metastasis was found. Immunohistochemically the tumor was shown to be positive for adrenocorticotropic hormone (ACTH), cytokeratin (KL1), neuron-specific enolase, synaptophysin, chromogranin and glucagon. Remarkably the tumour was negative for serotonin despite high urinary hydroxyindolacetic acid levels. Bilateral hyperplasia of the adrenal cortex was found. The adenohypophysis showed a considerable reduction of ACTH-producing cells and numerous Crooke's cells with a characteristic immunohistochemical pattern.

Adrenal Cortex↗
PubMed1992

[Value of "non-specific" lymphoid infiltrates in bone marrow in high grade malignant peripheral non-Hodgkin's lymphomas].

We studied iliac crest biopsies of 120 patients with extramedullary high-grade Non-Hodgkin's lymphomas (NHL). The bone marrow of 69 patients contained foci of small lymphoid cells, which cytologically differed significantly from the extramedullary NHL and which we termed abnormal lymphoid infiltrates (ALI). The ALIs in 14 out of 25 cases immunohistochemically investigated in more detail were made up of small CD3+ cells optionally intermingled with a minority of CD20+ cells of small to intermediate size. In 10 patients, however, the ALIs satisfied the criteria of a low-grade NHL. In the vicinity of the extramedullary high-grade lymphoma, residues of the corresponding low-grade NHL were identified in up to 7 of 10 cases. We conclude that bone marrow ALIs associated with extramedullary high-grade NHL exhibit at least in part the criteria of a systemic clonal expansion of premalignant lymphoid cells or low-grade NHLs respectively, which gives rise to the final evolution of the extramedullary high-grade NHL.

Antigens, CD↗
PubMed1991

Histopathological studies on the local reactions induced by complete Freund's adjuvant (CFA), bacterial lipopolysaccharide (LPS), and synthetic lipopeptide (P3C) conjugates.

The inflammatory reactions following subcutaneous application of adjuvants revealed characteristic pathological patterns. The injection of complete Freund's adjuvant (CFA) resulted in the formation of large lipid deposits encircled by an inflammatory reaction and concentrically arranged collagen bundles. Bacterial lipopolysaccharide (LPS) caused granulomatous aggregations of mononuclear cells with thrombotic vessel occlusions. Inoculation of the lipopeptide adjuvants induced accumulation of mononuclear cells with only minimal fibrotic changes which were resolved after day 28. Lipopeptide conjugates based on the head group tripalmitoyl-S-glyceryl-cysteinyl-serin (P3CS) can thus be used as effective immunogens and adjuvants without long-term tissue damage.

Animals↗
PubMed1991

Comparison of host responses after intranasal infection of guinea-pigs with Mycoplasma genitalium or with Mycoplasma pneumoniae.

Guinea-pigs were infected intranasally with Mycoplasma genitalium or Mycoplasma pneumoniae. The lung lesions produced by the two mycoplasmas were comparable in extent and histological pattern. Sera of both animal groups taken 2 weeks after infection reacted strongly in the complement fixation test with the M. pneumoniae glycolipid extract. In an ELISA using the respective adherence proteins (P1-protein of M. pneumoniae and MgPa of M. genitalium), strong specific activity, but also considerable cross-reactions were found. Epitope analysis by using overlapping octapeptides of a P1-region immunologically active in human M. pneumoniae infections and of the corresponding MgPa-region revealed six common epitopes but also one M. genitalium and two M. pneumoniae specific determinants. For analysis of a possible pathogenicity of M. genitalium in the human respiratory tract species-specific tests have to be developed.

Administration, Intranasal↗
PubMed1991

[Tumors in immunodeficiency syndromes].

It is the purpose of this review to analyse prevalence and pathogenesis of tumours occurring in the diverse conditions of primary and secondary immunodeficiencies. In general, most states of immunodeficiences are connected with a highly increased tumour risk. However, there is circumstantial evidence that immunodeficiency-related malignancies do not primarily result from an impaired immunosurveillance. With special regard to ataxia-telangiectasia it has been found that congenital chromosomal instability deregulates the maturation of immunocompetent lymphoid cells by interfering with their natural DNA rearrangements and eventually leads to false recombinatory events with oncogenetic consequences in a parallel way. Malignancies in primary common variable or in secondary (e.g. AIDS) immunodeficiency may result from an impaired immunity to oncogenic viruses or from chronic immune damage due to secondary autoimmune disorders.

Acquired Immunodeficiency Syndrome↗
PubMed1991

Immunohistochemical investigation of pericytes in chronic venous insufficiency.

Patients with chronic venous insufficiency show typical glomerulum like alterations of cutaneous capillaries. Objective of this study was to determine any changes of the alignment of pericytes around cutaneous capillaries in CVI patients. Skin biopsies from the area of the medial malleolus were taken from 42 patients with CVI, 5 healthy individuals and 11 cadavers without history of CVI. Sections were stained with HHF35, anti alpha and gamma muscle actin with the avidin-biotin-peroxidase method (ABC) and anti vimentin with the alkaline phosphatase anti-alkaline phosphatase technique (APAAP). The stage of stasis dermatosis was assessed and sections were examined for pericyte changes. Among the collective of 42 patients with CVI, 31 patients showed slight or severe pericyte changes, 11 patients were without changes. None of the sections from cadavers or healthy patients showed any pericyte changes. Pericytes are among other functions possibly involved in microvasculature regulation and wound healing. Thus destruction of the pericyte envelope might lead to microcirculatory dysfunction. This could be one of the causes that lead to leg ulcers in CVI.

Actins↗
PubMed1990

The occurrence of beta-glucuronidase in erythrocyte inclusions.

This study presents erythrocytic inclusion bodies exhibiting histochemically a strong beta-glucuronidase activity. The unique occurrence of this enzyme in cells of the erythropoietic series has never been described before and characterizes a novel type of erythrocyte inclusions mainly observed in patients suffering from liver damage but also from myelodysplasia and congenital dyserythropoietic anemia type I. Since it is known that hepatocytes contain high activities of beta-glucuronidase, we supposed a relationship between an increased breakdown of liver cells and the appearance of beta-glucuronidase positive inclusions in the red cells. To prove this hypothesis, we determined the beta-glucuronidase plasma levels of 99 unselected patients suffering from various liver disorders in comparison with 19 healthy controls by use of the umbelliferone technique. Our results indicate that in cases with liver diseases beta-glucuronidase plasma levels are significantly increased as compared with normal persons. The availability of a sufficient amount of beta-glucuronidase, thus, seems to be one prerequisite for the appearance of these inclusions in erythrocytes. As demonstrated by our investigations on congenital dyserythropoietic anemia, in which the red cells also show beta-glucuronidase positive inclusions, another premise seems to be an elevated autophagolysosomal activity in the erythrocytes.

Adult↗