Two Unrelated Burkitt Lymphomas Seven Years Apart in a Patient With X-Linked Lymphoproliferative Disease Type 1 (XLP1).
OBJECTIVE
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.


METHODS
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.


RESULTS
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.


CONCLUSIONS
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.
From the Department of Pathology, University of Utah, Salt Lake City.
Delu
D
Zhou
ARUP Laboratories, Salt Lake City, UT.
Christian N
CN
Paxton
From the Department of Pathology, University of Utah, Salt Lake City ARUP Laboratories, Salt Lake City, UT.
Todd W
TW
Kelley
Department of Pediatrics, University of Utah and Primary Children's Hospital, Salt Lake City.
Zeinab
Z
Afify
From the Department of Pathology, University of Utah, Salt Lake City ARUP Laboratories, Salt Lake City, UT.
Sarah T
ST
South
From the Department of Pathology, University of Utah, Salt Lake City ARUP Laboratories, Salt Lake City, UT Huntsman Cancer Institute, Salt Lake City, UT. rodney.miles@path.utah.edu.
Rodney R
RR
Miles
D000293:Adolescent; D014408:Biomarkers, Tumor / Q000032:analysis; D002051:Burkitt Lymphoma / Q000235:genetics / Q000473:pathology*; D002648:Child; D005434:Flow Cytometry; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D008232:Lymphoproliferative Disorders / Q000150:complications*; D008297:Male; D016609:Neoplasms, Second Primary / Q000235:genetics / Q000473:pathology*
D014408:Biomarkers, Tumor
B-cell clonality; Burkitt lymphoma; SNP array analysis; X-linked lymphoproliferative disease type 1