Related Experiment Videos
De novo CD5+ Burkitt lymphoma/leukemia
1Department of Laboratory Medicine, University of Texas M. D. Anderson Cancer Center, Houston 77030, USA.
American Journal of Clinical Pathology
|December 10, 1999
Summary
This study identifies rare cases of CD5-positive Burkitt lymphoma/leukemia in elderly patients. These aggressive lymphomas present unique diagnostic challenges due to similarities with mantle cell lymphoma.
Area of Science:
- Hematology
- Oncology
- Immunophenotyping
Background:
- CD5 is a T-cell marker typically absent in Burkitt lymphoma but present in B-cell chronic lymphocytic leukemia and mantle cell lymphoma.
- Burkitt lymphoma is an aggressive B-cell neoplasm usually characterized by c-myc rearrangement.
Observation:
- Four elderly patients presented with de novo CD5-positive Burkitt lymphoma/leukemia in leukemic phase.
- Morphologically, the blasts were medium-sized with folded nuclei and basophilic cytoplasm, negative for terminal deoxynucleotidyl transferase and positive for surface immunoglobulin.
- All cases showed c-myc rearrangement, lacking t(14;18), t(11;14), or cyclin D1 alterations.
Findings:
- The identified cases are classified as Burkitt leukemia due to molecular features (c-myc rearrangement) despite CD5 positivity.
- Treatment with hyper-CVAD (hyperfractionated cyclophosphamide, vincristine, doxorubicin, dexamethasone) showed limited efficacy, with only one patient achieving complete remission.
Implications:
- Distinguishing CD5+ Burkitt leukemia from the blastoid variant of mantle cell lymphoma is crucial for accurate diagnosis and treatment.
- Genetic analysis, specifically identifying c-myc rearrangement versus cyclin D1 alterations, is key to differentiating these entities.
- The poor response to standard chemotherapy highlights the need for further research into optimal therapeutic strategies for this rare lymphoma subtype.