- Am J Clin Pathol. 1999 Dec;112(6):828-35.
- De novo CD5+ Burkitt lymphoma/leukemia.
Lin CW, O'Brien S, Faber J, Manshouri T, Romaguera J, Huh YO, Kantarjian H, Keating M,
Albitar M.
Department of Laboratory Medicine, University of Texas M. D. Anderson Cancer Center,
Houston 77030, USA.
CD5 is a T-cell marker aberrantly expressed in B-cell chronic lymphocytic leukemia and
mantle cell lymphoma. Other B-cell neoplasms, including Burkitt lymphoma, are usually
CD5-. We report 4 cases of de novo CD5+ Burkitt lymphoma/leukemia in elderly patients, all
of whom were in a leukemic phase and had variable lymph node and splenic involvement. The
blasts were typically medium sized, with folded nuclei, distinct but not prominent
nucleoli, and moderate amounts of somewhat vacuolated basophilic cytoplasm; they were
terminal deoxynucleotidyl transferase--negative and surface immunoglobulin--positive. All
4 cases demonstrated c-myc rearrangement, but none had t(14;18), t(11;14), or cyclin D1
overexpression or rearrangement. Only 1 patient achieved complete remission after
hyper-CVAD (hyperfractionated cyclophosphamide, vincristine, doxorubicin, dexamethasone)
therapy. One patient responded poorly to hyper-CVAD, and 2 patients died during induction
chemotherapy. These rare cases of aggressive lymphoid malignancy with CD5 positivity and
molecular features associated with Burkitt lymphoma/leukemia are best classified as
Burkitt leukemia. However, the morphologic and immunophenotypic similarity to the blastoid
variant of mantle cell lymphoma are diagnostically challenging. The diseases can be
distinguished at the genetic level, since Burkitt lymphoma involves the rearrangement of
c-myc, and mantle cell lymphoma usually the overexpression or rearrangement of cyclin D1.
PMID: 10587706 [PubMed - indexed for MEDLINE]