Epstein-Barr-associated leiomyomatosis and T-cell chimerism after haploidentical bone marrow transplantation for severe combined immunodeficiency disease.


Journal Article

BACKGROUND: The clinical course of Epstein-Barr virus (EBV)-associated smooth muscle tumors is variable and there are no reports in patients with mixed T-cell chimerism after bone marrow transplantation (BMT). OBSERVATIONS: A child with X-linked severe combined immunodeficiency disease developed multiple renal and pulmonary leiomyomata 8 years after haploidentical BMT. Epstein-Barr viral DNA was detectable in the blood and in situ hybridization for EBV-encoded RNAs was positive in the tumor. The tumors have been radiographically stable, chimerism remains mixed, and plasma EBV DNA has been repeatedly negative for over 2 years after donor lymphocyte infusion. CONCLUSIONS: EBV-associated smooth muscle tumors may occur in patients who are partially reconstituted after BMT for severe combined immunodeficiency and may not require surgery or chemotherapy.

Full Text

Duke Authors

Cited Authors

  • Atluri, S; Neville, K; Davis, M; Robertson, KA; Marshalleck, FE; O'Malley, DP; Buckley, RH; Nelson, RP

Published Date

  • March 2007

Published In

Volume / Issue

  • 29 / 3

Start / End Page

  • 166 - 172

PubMed ID

  • 17356396

Pubmed Central ID

  • 17356396

International Standard Serial Number (ISSN)

  • 1077-4114

Digital Object Identifier (DOI)

  • 10.1097/MPH.0b013e31803b95b3


  • eng

Conference Location

  • United States