Unusual clinical and immunologic manifestations of transplacentally acquired maternal T cells in severe combined immunodeficiency
| Autor: | Roberta E. Parrott, Todd D. Green, Dong-Feng Chen, Kricia Palmer, Rebecca H. Buckley, Joseph L. Roberts, Nancy L. Reinsmoen, E.O. Sajaroff, Myriah Cooney |
|---|---|
| Rok vydání: | 2007 |
| Předmět: |
Reoperation
CD3 Complex Pancytopenia T-Lymphocytes Lymphocyte Immunology Autoimmune Diseases Antibodies Monoclonal Murine-Derived Immune system medicine Humans Immunologic Factors Immunology and Allergy Lymphocytes Bone Marrow Transplantation Cell Proliferation Severe combined immunodeficiency Receptors Interleukin-7 business.industry Janus kinase 3 Antibodies Monoclonal Infant Janus Kinase 3 T lymphocyte medicine.disease Virology Transplantation medicine.anatomical_structure Graft-versus-host disease Haplotypes Leukocyte Common Antigens Female Severe Combined Immunodeficiency Mitogens Rituximab business Immunity Maternally-Acquired |
| Zdroj: | Journal of Allergy and Clinical Immunology. 120:423-428 |
| ISSN: | 0091-6749 |
| DOI: | 10.1016/j.jaci.2007.02.047 |
| Popis: | The persistence of transplacentally transferred maternal T cells is common in infants with severe combined immunodeficiency (SCID), occurring in more than half of patients with SCID undergoing transplantation at our institution. These T cells respond poorly to mitogens in vitro but can cause cutaneous graft-versus-host disease; however, other effects of these cells are unknown. We describe 2 infants with SCID who had unusual problems associated with transplacentally transferred maternal T cells. Patient 1 was a 5-month-old girl with Janus kinase 3-deficient SCID who had 4% circulating CD3(+) T cells but no lymphocyte proliferative response to mitogens. Although the number of T cells increased after 2 nonchemoablated, T cell-depleted, haploidentical, paternal bone marrow transplantations, T-cell function failed to develop, and she became pancytopenic. Restriction fragment length polymorphism studies of flow cytometry-sorted blood T cells revealed all to be of maternal origin. A subsequent nonchemoablated, T cell-depleted maternal transplantation resulted in normal T-cell function and marrow recovery. Patient 2 was a 9-month-old girl with IL-7Ralpha-deficient SCID who presented with autoimmune pancytopenia. She had 8% blood T cells (all CD45RO(+)) but no response to mitogens. High-resolution HLA sequence-specific priming typing detected both maternal haplotypes, indicating the presence of maternal cells. Her pancytopenia resolved after treatment with rituximab and was thought to be due to host B-cell activation by transplacentally acquired maternal T cells. Persistent transplacentally acquired maternal T cells in infants with SCID can mediate immunologic functions despite failing to respond to mitogens in vitro. We present evidence that these cells can cause allograft rejection and immune cytopenias. |
| Databáze: | OpenAIRE |
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