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

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.

• Authors: Srilatha Atluri, Kathleen Neville, Mary Davis, Kent A Robertson, Francis E Marshalleck, Dennis P O'Malley, Rebecca H Buckley, Robert P Nelson Jr
• Journal: Journal of pediatric hematology/oncology (J Pediatr Hematol Oncol) Vol. 29 Issue 3 Pg. 166-72 (Mar 2007) ISSN: 1077-4114 [Print] United States
• PMID: 17356396 (Publication Type: Case Reports, Journal Article)

Topics

• Bone Marrow Transplantation (adverse effects, immunology)
• Child
• Chimerism
• Epstein-Barr Virus Infections (complications, pathology)
• Humans
• Kidney Neoplasms (diagnosis, virology)
• Leiomyomatosis (pathology, virology)
• Lung Neoplasms (diagnosis, virology)
• Male
• Sensitivity and Specificity
• Severe Combined Immunodeficiency (immunology, therapy, virology)
• T-Lymphocytes (pathology, virology)
• Tomography, X-Ray Computed (methods)