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- Title
Two Unrelated Burkitt Lymphomas Seven Years Apart in a Patient With X-Linked Lymphoproliferative Disease Type 1 (XLP1).
- Authors
Zhou, Delu; Paxton, Christian N.; Kelley, Todd W.; Afify, Zeinab; South, Sarah T.; Miles, Rodney R.
- Abstract
<bold>Objectives: </bold>We describe a rare case of a male child with X-linked lymphoproliferative disease type 1 (XLP1) who presented with Burkitt lymphoma (BL) when he was 6 years old, achieved a complete response to therapy, and developed a second BL after seven years.<bold>Methods: </bold>Diagnostic H&E stained slides and ancillary studies were reviewed for both lymphomas. B-cell clonality by PCR and SNP array studies were performed on both specimens.<bold>Results: </bold>Both lymphomas were Epstein-Barr virus (EBV) negative. Flow cytometry showed λ light chain restriction in the initial BL and κ light chain restriction in the subsequent BL. B-cell clonality testing indicated that the two lymphomas are not clonally related. SNP array analysis of the second BL showed genomic changes that were not present in the first BL.<bold>Conclusions: </bold>These results confirm that these two tumors represent unrelated BLs. Pathologists and clinicians should be aware that second lymphomas in XLP1 patients may represent new neoplasms rather than late relapses.
- Subjects
LYMPHOPROLIFERATIVE disorders; BURKITT'S lymphoma; EPSTEIN-Barr virus; FLOW cytometry; LYMPHOMAS; B cell lymphoma; FLUORESCENCE in situ hybridization; SECONDARY primary cancer; DISEASE complications
- Publication
American Journal of Clinical Pathology, 2016, Vol 146, Issue 2, p248
- ISSN
0002-9173
- Publication type
journal article
- DOI
10.1093/ajcp/aqw036