Unusual clinical and immunologic manifestations of transplacentally acquired maternal T cells in severe combined immunodeficiency

JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY

卷:120

Article

Palmer, Kricia ; Green, Todd D. ; Roberts, Joseph L. ; Sajaroff, Elisa ; Cooney, Myriah ; Parrott, Roberta ; Chen, Dong-Feng ; Reinsmoen, Nancy L. ; Buckley, Rebecca H.

关键词: severe combined immunodeficiency;    transplacentally transferred maternal T cells;    graft-versus-host disease;   

DOI  :  10.1016/j.jaci.2007.02.047

来源: Elsevier

【 摘 要 】

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 How 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-7R alpha-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 respond to mitogens in vitro. We present evidence that these cells can cause allograft rejection and immune cytopenias.

【 授权许可】

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