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Biomedical subjects

S F Goldmann

Publications and source records attributed to S F Goldmann.

At least 91 records · Page 5Linked to original sources

Congenital immunodeficiency and agranulocytosis (reticular dysgenesia).

A patient is presented who manifested the typical clinical and pathological features of congenital immunodeficiency and agranulocytosis (reticular dysgenesia). Treatment under gnotobiotic conditions enabled the measurement of immunogical parameters up to the 17th week of life with the following results: negative skin test, low response to phytohaemagglutinin, weak response in the mixed leukocyte culture and very few E rosettes. Peripheral lymphocytes and lymphocytes in the lymphatic tissues were markedly decreased. Humoral immunoglobulins and plasma cells in the organs were decreased. The in vitro culture of hemopoietic cells showed a diminished content of myelopoietic progenitor cells ("committed stem cells"). It is concluded that the disease may be primarily a defect of stem cells with regard to differentiation in myelopoiesis or lymphopoiesis.

Agranulocytosis↗

Education of lymphocytes in vivo following a transient take of a matched unrelated bone-marrow graft in man.

A patient suffering from aplastic anaemia was treated by bone-marrow transplantation from an ABO- and HLA-identical, MLC- and CML-negative, unrelated donor. MLC and CML became positive after transplantation indicating that a cellular immune response had developed against lymphocyte determinants not recognized prior to sensitization in vivo. Whether these determinants are governed by genes of the HLA region is unknown at present.

Adult↗

High dose granulocyte substitution after bone marrow transplantation in a case of aplastic anaemia in childhood.

A bone marrow transplantation (BMT) was successfully carried out in a seven-year-old boy with aplastic anaemia. An HLA and MLC-identical brother served as donor. During the first 16 days after BMT between 0.55 and 7.11X10(10) granulocytes were transfused 14 times (an average of 4.03X10(10) per transfusion). The maximum increment in the peripheral blood after transfusion was over 4000 granulocytes per microliter. The course of local infections was taken as parameter for determining the clinical effectiveness of the granulocyte transfusion. Under the substitution therapy the necrotic ulcer of a fingertip and the mucous membrane ulcers in the oral cavity subsided. The presence of transfused granulocytes in the oral cavity ("orogranulocytes") could be established. There was a statistically significant relationship between the number of granulocytes in the peripheral blood and the number of orogranulocytes. It could be demonstrated that in children a temporary normalisation of the number of granulocytes in the peripheral blood can be attained by means of granulocyte substitution and that the transfused granulocytes are clinically effective.

Anemia, Aplastic↗

[Supportive care in bone marrow failure. Technical, immunological and clinical advances].

Supportive care with blood component transfusions has greatly improved prognosis in patients with bone marrow failure. This progress has made possible by newly developed techniques for separation of blood cells and by a better understanding of the antigenicity of human blood cells and of immunologic reactions following their transfusion. Transfusion of white cell and platelet-poor red cell preparations prevent alloimmunization to leukocyte and platelet-bound alloantigens, or non-hemolytic transfusion reactions in already alloimmunized patients. Alloimmunization can be circumvened and effective long-term platelet support to thrombocytopenic patients can be provided by matching donor and recipient for HL-A antigens. The place of granulocyte transfusion in clinical therapy has yet to be defined, although their usefulness in infected granulocytopenic patients is suggested by the few studies reported so far.

Antibody Formation↗

Bone marrow transplantation for aplastic anaemia from a HL-A and MLC-identical unrelated donor.

Bone-marrow transplantation (BMT) from an unrelated, HL-A-phenotype-identical, MLC-negative donor was performed in a 31 year old woman with severe longlasting aplastic anemia. In vitro assays failed to demonstrate humoral or cellular sensitization of the recipient against donor-type antigens. Following conditioning with cyclophosphamide, prompt but only transient engraftment of the transplant occurred accompanied by signs of mild graft-versus-host-disease (GVHD) of the liver. The results of a second bone marrow transplantation from the same donor cannot be evaluated due to early death of the recipient. It is concluded that bone marrow from unrelated, HL-A and MLC-identical donors may engraft without severe GVHD. Rejection of the graft in our patient may have been related to greater antigenic differences that can be expected to exist between HL-A and MLC-identical unrelated individuals than between HL-A and MLC-identical siblings. However, insufficient preparative immunosuppression with cyclophosphamide due to severe hepatic hemosiderosis appears equally likely as the cause of graft rejection. The possibly increased risk of graft rejection or severe GVHD should not preclude the use of unrelated HL-A and MLC-identical marrow donors, when histocompatible sibling donors are not available; but more potent immunosuppressive regimens than the cyclophosphamide protocol may be necessary to ensure permanent engraftment.

Adult↗

Histocompatibility testing in dogs. II. Leukocyte typing in relation to the mixed lymphocyte culture (MLC) reactivity.

The correlation between MLC reactivity (LD) and serological leukocyte typing (SD) was studied in a beagle colony. Disparity for a serologically defined non-DL-A lymphocyte antigen did not correlate with MLC reactivity. Lymphocytes of colony members with common ancestors and SD identical DL-A haplotypes did not stimulate each other in the MLC. This implies that LD typing in the beagle coolony can be generally predicted by DL-A SD typing. Consequently, lymphocytes of sibs homozygous for a given DL-A SD haplotype could be shown, with few exceptions, to be also homozygous for MLC determinants. Cells of these homozygous sibs can be used in MLC typing as reference cells for DL-A LD specificities. Two exceptions to the expected linkage between DL-A SD typing and MLC reactivity were found. These findings could not be explained by recombination with the DL-A region assuming a single major LD locus coding for MLC. Thus, suggestive evidence for more than one single LD locus has been obtained.

Animals↗

Histocompatibility testing in dogs. I. A semi-micro mixed lymphocyte culture (MLC) technique for histocompatibility matching in dogs.

A semi-micro method for the mixed lymphocyte culture (MLC) test in dogs is described. Optimization of various factors influencing the test were invistigated. Discrimination between allogeneic and isogeneic cell mixtures was possible after 90 h or culture but a culture period of 144 h was found to be optimal. The method measures MLC identity or non-identity and can be used as a tool for histocompatibility testing of lymphocyte defined (LD) Antigens.

Animals↗