Epstein-Barr-associated leiomyomatosis and T-cell chimerism after haploidentical bone marrow transplantation for severe combined immunodeficiency disease.
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.
Duke Scholars
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Related Subject Headings
- Tomography, X-Ray Computed
- T-Lymphocytes
- Severe Combined Immunodeficiency
- Sensitivity and Specificity
- Oncology & Carcinogenesis
- Male
- Lung Neoplasms
- Leiomyomatosis
- Kidney Neoplasms
- Humans
Citation
Published In
DOI
ISSN
Publication Date
Volume
Issue
Start / End Page
Location
Related Subject Headings
- Tomography, X-Ray Computed
- T-Lymphocytes
- Severe Combined Immunodeficiency
- Sensitivity and Specificity
- Oncology & Carcinogenesis
- Male
- Lung Neoplasms
- Leiomyomatosis
- Kidney Neoplasms
- Humans