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

Srilatha Atluri, Kathleen Neville, Mary Davis, Kent A. Robertson, Francis E. Marshalleck, Dennis P. O'Malley, Rebecca H. Buckley, Robert P. Nelson

Research output: Contribution to journalArticle

12 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)166-172
Number of pages7
JournalJournal of Pediatric Hematology/Oncology
Volume29
Issue number3
DOIs
StatePublished - Mar 1 2007

Keywords

  • Donor lymphocyte infusion
  • EBV-associated leiomyomatosis
  • Mixed chimerism
  • Severe combined immunodeficiency disease

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Oncology
  • Hematology

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