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).


METHODS
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.
Atluri
Srilatha
S
Department of Medicine, Indiana University School of Medicine, Indianapolis, IN 46202, USA.
Neville
Kathleen
K
Davis
Mary
M
Robertson
Kent A
KA
Marshalleck
Francis E
FE
O'Malley
Dennis P
DP
Buckley
Rebecca H
RH
Nelson
Robert P
RP
D016026:Bone Marrow Transplantation / Q000009:adverse effects* / Q000276:immunology; D002648:Child; D046528:Chimerism; D020031:Epstein-Barr Virus Infections / Q000150:complications* / Q000473:pathology; D006801:Humans; D007680:Kidney Neoplasms / Q000175:diagnosis* / Q000821:virology; D018231:Leiomyomatosis / Q000473:pathology* / Q000821:virology; D008175:Lung Neoplasms / Q000175:diagnosis* / Q000821:virology; D008297:Male; D012680:Sensitivity and Specificity; D016511:Severe Combined Immunodeficiency / Q000276:immunology / Q000628:therapy* / Q000821:virology; D013601:T-Lymphocytes / Q000473:pathology* / Q000821:virology; D014057:Tomography, X-Ray Computed / Q000379:methods