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J Pediatr Hematol Oncol. 2008 May;30(5):396-400. doi: 10.1097/MPH.0b013e318168e7a0.

Maternal T-cell engraftment associated with severe hemophagocytosis of the bone marrow in untreated X-linked severe combined immunodeficiency.

Author information

1
Division of Pediatric Stem Cell Transplantation, Department of Pediatrics, Stanford University School of Medicine, Stanford, CA, USA. dvorakc@peds.ucsf.edu

Abstract

Maternal engraftment of T cells in severe combined immunodeficiency can lead to graft-versus-host disease of the skin and liver. We report the case of an infant with X-linked severe combined immunodeficiency, confirmed by DNA sequencing of the common gamma chain gene locus, in which this disorder's characteristic peripheral lymphocyte phenotype [T(-)B(+)NK(-)] was obscured by the postnatal onset of hemophagocytic syndrome that included severe B-cell lymphopenia, neutropenia, and anemia. Hemophagocytosis was most likely owing to maternal graft-versus-host disease, as perforin-expressing CD8 T cells, presumably of maternal origin, were prominent in the bone marrow and there was no concurrent severe infection.

PMID:
18458578
DOI:
10.1097/MPH.0b013e318168e7a0
[Indexed for MEDLINE]

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