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Biomedical subjects

W Friedrich

Publications and source records attributed to W Friedrich.

At least 91 records · Page 5Linked to original sources
PubMed1991

Coexistence of donor and host T lymphocytes following HLA-different bone marrow transplantation into a patient with cellular immunodeficiency and nonfunctional CD4+ T cells.

We report the outcome of a non-T-cell-depleted bone marrow transplant from an HLA partially incompatible, MLR-positive, parental donor in a patient with an unusual form of immunodeficiency characterized by a lack of CD8 T cells and a failure of the CD4 cells to display functional activity in vitro. Without conditioning, and following a mild and transient GVHD, donor T cells persist in trace amounts in the host, where they coexist with the nonfunctional host T cells and cooperate with host APC in antigen recognition, thereby leading to a reconstitution of T cell functions in vitro and in vivo and development of a stable, so far unprecedented, human T-T split chimera across MHC barriers.

Antigens, Differentiation, T-Lymphocyte↗
PubMed1991

[Pathogenesis and histomorphology of the so-called Omenn syndrome].

1. The Omen-syndrome is not a disease on its own, but a complication of congenital SCID. 2. In contrast to patients with classical SCID, patients with Omenn-syndrome possess mature T-cells, which are either of maternal or of host origin. 3. These T-cells are involved in the pathogenesis of the characteristic tissue changes, in particular of skin and lymph nodes (Langerhans-histiocytosis with eosinophilia). 4. The detection of immunodeficiency in Omenn-syndrome is difficult since the lymph nodes are enlarged in contrast to patients with classical SCID. The histomorphological analysis of lymph nodes in Omenn-syndrome is considerably complicated by secondary changes closely resembling dermatopathic lymphadenopathia.

Eosinophils↗
PubMed1991

[Histomorphology of BCG infections in patients with severe combined immunodeficiency].

Most inborn immunodeficiency syndromes clinically become overt by complicating infections, e.g. by BCG-infection after BCG-vaccination. The diagnostic approach often is hampered by an atypical histomorphological picture of the infection caused by the underlying immune defect. We examined biopsies of different organs in 18 infants with severe combined immunodeficiency syndrome and BCG-infection. A number of unusual histomorphological patterns of BCG-infection were found not yet published previously. There was a correlation between histomorphological features and immunological data or clinical course in most cases.

Adenosine Deaminase↗
PubMed1991

T cell receptor diversity in severe combined immunodeficiency following HLA-haploidentical bone marrow transplantation.

We have studied T cell receptor (TCR) diversity in a group of six patients with severe combined immunodeficiency (SCID) previously treated by HLA-haploidentical bone marrow transplantation (BMT). At the time of study, all patients had developed stable T cell chimerism and full reconstitution of T cell functions in the absence of acute or chronic graft-versus-host disease. Peripheral blood lymphocytes (PBL) were analysed by immunofluorescence using a panel of monoclonal antibodies (MoAb) against TCR variable (V) region epitopes including V beta 5, V beta 6, V beta 8, V beta 12, and V alpha 2. Our results showed that in each patient studied a low but significant portion of PBL reacted with each anti-TCR V region epitope MoAb used, in a manner that was, on statistical grounds, indistinguishable from results obtained with PBL from healthy controls. We conclude that within the experimental resolution of a limited number of anti-TCR V region epitope MoAb, T cell reconstitution following BMT for SCID, even when performed across a full HLA-haplotype barrier, leads to an apparently normal TCR diversity. These novel findings may be relevant in the evaluation of functional capacities of T cells that have differentiated from transplanted precursor cells in an HLA-haplodifferent environment.

Bone Marrow Transplantation↗
PubMed1990

European experience of bone-marrow transplantation for severe combined immunodeficiency.

The outcome of bone-marrow transplantations (BMT) carried out between 1968 and March 1, 1989, in 183 patients with severe combined immunodeficiency (SCID) was analysed. Recipients of HLA-identical BMTs (70) had a 76% probability of survival (median follow-up 73 months). Of the 32 treated since 1983, 97% have been cured (median follow-up 41 months). This good prognosis was associated with rapid development of T and B cell function. HLA-non-identical, T-cell-depleted, BMT (n = 100) gave significantly lower survival (52%; median follow-up 47 months). Factors associated with poor prognosis were the presence of a lung infection before BMT, absence of a protected environment, and use of female donors for male recipients. Use of a conditioning regimen significantly increased the frequency of sustained engraftment (86% vs 50% for non-conditioned BMT) and resulted in more frequent engraftment of donor B lymphocytes and myeloid cells. Donor B-cell chimerism was strongly associated with the development of normal B-cell function.

Actuarial Analysis↗
PubMed1990

[Miescher's granulomatous cheilitis. Diagnostic and therapeutic aspects].

Granulomatous cheilitis Miescher is a rare condition of unclear etiology, which is discussed as a monosymptomatic feature of Melkersson-Rosenthal-Syndrome, an extraintestinal form of Crohn's Disease or an unspecific cutaneous symptom of any granulomatous disease. Clinical appearance and diagnostic aspects are described. Therapeutical approaches as surgical intervention, corticoid- and sulfasalazine therapy and especially efforts with systemic clofazimine treatment are discussed.

Adrenal Cortex Hormones↗
PubMed1990

[Reticular dysgenesis: primary disorder in differentiation of hematopoietic stem cells?].

Reticular Dysgenesis (RD) basically represents a lymphopenic severe combined immunodeficiency (SCID) in association with congenital agranulocytosis. It is presumed that RD results from a primary defect of pluripotent hematopoietic stem cells (HSC). Alternatively RD might be due to an alloreaction induced by T-cells derived from intrauterine transfusion of maternal cells into an immunoincompetent host. In the past 15 years, among 49 newborns with SCID taken care of in the University Hospital of Ulm, 5 children (4 boys, 1 girl) exhibited the characteristics of RD. In 3 of 4 cases studied, maternal T-cells were detected by HLA-typing. All 3 children showed signs of graft-versus-host-disease (GvHD), confirmed histologically. However, 9 of 45 newborns with SCID without congenital agranulocytosis also disclosed maternal T-cell-engraftment; 5 of the 9 had signs of GvHD. Therefore, it is unlikely that RD is caused by GvHD secondary to maternofetal transfusion. The fact that erythropoiesis, thrombopoiesis and the monocyte-macrophage-system basically are intact argues against a global maturation defect of HSC in RD.

Bone Marrow↗
PubMed1990

[Quantitative skin test and inhalation provocation test in asthma using platinum salts].

In this article, the in vivo test results obtained from workers of two platinum separating plants are described. A total of 30 out of 102 workers in two platinum separating plants and 0/40 control subjects showed a positive skin reaction on being provoked with platinum compounds. Twelve out of 15 workers and none of 5 control subjects showed a positive reaction to the bronchial provocation test; this indicates that both tests are highly specific. Three workers with negative skin test reactions showed a clearly positive immediate reaction to the bronchial provocation test with platinum compounds.

Bronchial Provocation Tests↗
PubMed1990

T cell depletion from human bone marrow using magnetic beads.

We have studied a recently described method to purge human marrow of T cells using magnetic beads conjugated to various anti-T cell monoclonal antibodies. Using anti-CD2 and anti-T cell receptor/CD3 monoclonal antibodies, we show that up to 2.76 log T cell purge can be achieved while recovery of hemopoietic activity is 82%. As a control, our clinically established T cell depletion protocol, namely soybean agglutination combined with E-rosetting, provided 3.39 log T cell purge and 59% recovery of hemopoietic activity. The impact of these results on T cell depletion strategies in HLA-nonidentical bone marrow transplantation is discussed.

Bone Marrow↗
PubMed1989

HLA-haploidentical bone marrow transplantation in three infants with adenosine deaminase deficiency: stable immunological reconstitution and reversal of skeletal abnormalities.

Three infants with severe combined immunodeficiency and adenosine deaminase (ADA) deficiency were treated by T-cell depleted bone marrow transplantation (BMT), using human leukocyte antigen (HLA)-haploidentical parents as donors. In the first patient, two initial transplants failed to engraft and no change of the immunodeficiency was observed. In order to overcome this graft resistance, cytoreductive conditioning was used prior to a third transplant. In the other two patients, similar conditioning was used prior to initial transplants. In all three patients, complete and permanent immunological reconstitution was observed and they survive from 3.5 to 5 years after transplantation. In biopsies obtained from iliac bones prior to BMT, osteochondral abnormalities characteristic of ADA-deficiency were noted in all three patients. After successful transplantation, these abnormalities had completely resolved. Our results demonstrate that cytoreductive conditioning prior to HLA-haploidentical BMT is useful in order to obtain stable engraftment and reversal of abnormalities associated with ADA deficiency.

Adenosine Deaminase↗
PubMed1988

Urinary neopterin in infants with primary immunodeficiency.

Neopterin is produced in large amounts specifically from macrophages upon stimulation with interferon-gamma (IFN-gamma). Measurement of neopterin allows a direct in vivo quantification of T cell activation. This is particularly useful, e.g., in early diagnosis of graft rejection. Since disease states with elevated neopterin levels in some cases are coupled with an impaired cellular immunity, we decided to investigate the possible influence that severely diminished cellular immunity might have on urinary neopterin levels. Our investigation on six children with severe primary immunodeficiency presents some evidence that immunodeficiency itself does not account for an increase in neopterin when patients are free from infections. Neopterin was also normal in an SCID patient who was completely lacking T cells and was suffering from severe infections. Two patients with primary immunodeficiency and residual T lymphocytes suffered from severe infections and showed elevated neopterin. The data support the hypothesis that elevated neopterin levels are dependent on the presence of activated T lymphocytes. Residual T lymphocytes of SCID patients have the capacity to induce neopterin in vivo when patients suffer from infections.

Adenosine Deaminase↗
PubMed1988

Asthma due to the complex salts of platinum--a cross-sectional survey of workers in a platinum refinery.

Anamnestic and immunological data of workers of a platinum refinery (group A: workers with work-related symptoms, n = 8; group B: workers with symptoms not clearly work-related, n = 9; group C: asymptomatic workers, n = 13) and controls (group D: atopics, n = 10; group E: non-atopics, n = 16) were compared. Exposure to platinum salt was higher in group A than in groups B or C. In group A, symptoms developed 4 months (median) after the onset of exposure. All subjects of group A and three workers of group B, but none of the workers of the other groups, showed a positive cutaneous reaction to (PtCl6)2-. Total serum IgE was higher in groups A and D than in groups B, C or E. (PtCl6)2(-)-specific IgE was higher in group A, but there was non-specific binding of (PtCl6)2- to IgE. Histamine release with (PtCl6)2- was found in all groups and was highest in atopic controls. Histamine release with (PtCl6)2- and histamine release with anti-IgE showed an excellent correlation, suggesting a similar release mechanism of (PtCl6)2- and anti-IgE. In skin-test positive subjects, high cutaneous (PtCl6)2(-)-sensitivity is linked to high histamine release with (PtCl6)2- or anti-IgE, supporting the concept of a role of cell surface IgE or IgE-Fc-receptor in the release process with platinum salts. However, high specificity of cutaneous reactions contrasts with low specificity of in-vitro tests with (PtCl6)2-. A different reaction of basophils and mast cells, when challenged with free platinum salts, is hypothesized. We conclude that neither histamine release from basophils with (PtCl6)2- nor RAST for the detection of (PtCl6)2- -specific IgE are helpful in the diagnosis of platinum salt allergy.

Asthma↗