Y Chromatin as Indicator of Chimaerism Following Bone Marrow Transplantation in Severe Combined Immunodeficiency

Are Ty cells of myelomonocytic lineage?To evaluate the cell lineage from which Ty cells derive by means other than cell membrane markers, the rare situation of human chimerism was studied. In patients with severe combined immunodeficiency who have been successfully treated by bone marrow transplantation, the occurrence of split take has been well documented: whereas myelomonocytic hemopoietic cell lines remain of host origin, the T lymphoid compartment is of donor origin, and the B lymphoid compartment may be either of host or of donor origin. We have studied the Ty cells of two patients successfully treated by bone marrow transplantation from donors of the opposite sex with respect to the sex chromosome pattern, the binding of OKMl and OKT3 monoclonal antibody and the killer (K) and natural killer (NK) cell activities. All Ty cells of both patients were found to be of donor origin. These Ty cells contained two serologically distinct subpopulations, one OKMl+, OKT3+, the other OKM1+, OKT3-, as was also found in normal persons. However, the ratio between these two subpopulations is < 1, whereas the ratio in these patients was > 1. Furthermore, the patients' Ty cells displayed strongly reduced K and NK activities (< 5% of normal). It was concluded that at least part of the OKMl', OKT3' and the OKMl', OKT3-Ty cells are derived from other than the myelomonocytic lineage, presumably from the lymphocytic lineage. The origin of the K-and NK-active Ty cells, however, cannot be conclusively determined from these experiments. The findings also imply that the antigen detected by OKMl should no longer be regarded as exclusively present on myelomonocytic cells.

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Are Tγ cells of myelomonocytic lineage?

Astaldi

Griend

Schellekens

et al. 1982

Eur J Immunol

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Bone marrow transplantation for severe combined immunodeficiency disease

Kenny

Hitzig

1979

Eur J Pediatr

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Patients who received bone marrow transplantation (= BMT) for the treatment of severe combined immunodeficiency (= SCID), and who were reported in the medical literature from 1968 to 1977, were collected and analysed. Eighteen of these 80 children are still alive, 10 months to 9 years after transplantation. It is thus the first successful form of therapy for this otherwise invariably fatal disease. Fifteen of the 18 survivors received bone marrow cells from HLA and MLC compatible donors; the remaining 3 survivors received grafts from MLC-compatible but HLA-incompatible donors. Bone marrow transplantation is the treatment of choice for SCID when recipient and donor are HLA- and MLC-identical. All patients who received MLC-incompatible grafts died, and bone marrow transplantation for SCID from MLC-incompatible donors should be abandoned. Milt-to-severe graft-versus-host disease (= GVHD) occurred in spite of HLA- and/or MLC-compatibility, with some correlation to the number of cells transplanted. This should preferably be kept below 50 million cells per kilo body weight. Infection was the chief cause of death in all groups. Strict reverse isolation, bowel decontamination and routine pre- and post-transplant Pneumocystis carinii prophylactic treatment are recommended. The clinical picture and laboratory findings of these 80 children before BMT did not differ from non-transplanted SCID patients. Three of the 18 survivors are adenosinedeaminase deficient.

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Recent Advances in Transplantation for Primary Immune Deficiency Diseases: A Comprehensive Review

Morena

Nelson

2013

Clinic Rev Allerg Immunol

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Hematopoietic cell transplantation (HCT) is a curative therapeutic option for severe combined immunodeficiency (SCID), a group of diseases which otherwise carry life expectancies that are of limited duration and quality. Survival following HCT for SCID has improved from approximately 23 to 91 % over the last 40 years. Success with SCID prompted efforts to apply HCT to the therapeutic challenge of well over 20 molecularly defined primary immune deficiency diseases (PID). Such success is due to both early recognition of PIDs and advances in the field of transplantation. Such advances include high-resolution HLA DNA donor-recipient matching, expansion of donor sources, better tolerated conditioning, new antibiotics, and wider availability. International collaborative efforts have provided patients and caregivers information that permit better treatment decisions now, and direct clinicians and investigators to ensure progress in the future. Pioneers in screening for SCID have taken steps to correct the fundamental challenge to successful treatment, which is the rapid discovery and characterization of cases and offering the transplant option to an affected child early in life; blood spot testing for T and B cell receptor quantification is now available to a growing fraction of newborns. Organizations including the Primary Immune Deficiency Treatment Consortium in the USA, The European Society for Primary Immunodeficiency, the European Group for Blood and Marrow Transplantation, the Pediatric Blood and Marrow Transplant Consortium, the United States Immunodeficiency Network, the Immune Deficiency Foundation, and the Jeffrey Modell Foundation are contributing mightily to increase awareness and standardize optimal utilization to the benefit of patients. This review will update the allergist-immunologist concerning disease presentations, indications for transplantation, methodologies, conditioning regimens, and clinical outcomes for patients with PID for which timely HCT is critical.

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Y Chromatin as Indicator of Chimaerism Following Bone Marrow Transplantation in Severe Combined Immunodeficiency

Cited by 4 publications

References 8 publications

Are Tγ cells of myelomonocytic lineage?

Are Tγ cells of myelomonocytic lineage?

Bone marrow transplantation for severe combined immunodeficiency disease

Recent Advances in Transplantation for Primary Immune Deficiency Diseases: A Comprehensive Review

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