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Unusual IgD+/CD38-follicular lymphoma with leukemic presentation
Grzegorz Rymkiewicz1, Ewa Paszkiewicz-Kozik, Katarzyna Blachnio
1Department of Pathology, Maria Sklodowska-Curie Memorial Cancer Centre, Warsaw, Poland. grzegorzrymkiewicz@coi.waw.pl
Medical Oncology (Northwood, London, England)
|April 29, 2006
Summary
Follicular lymphoma can rarely present as a leukemic phase. This case highlights a patient achieving complete remission with chemotherapy and rituximab despite extensive disease.
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- Follicular lymphoma (FL) is typically a low-grade non-Hodgkin lymphoma.
- Leukemic transformation of FL at diagnosis is a rare clinical presentation.
Observation:
- A 49-year-old woman presented with leukemic phase of FL 3 months after initial diagnosis of peripheral lymph node involvement.
- Diagnostic workup included flow cytometry, PCR for bcl-2 rearrangement, and cytogenetic analysis.
- Flow cytometry revealed lymphoma cells negative for CD38, expressing bright IgD and dim kappa-chains.
Findings:
- The patient had generalized lymphadenopathy and significant peripheral blood and bone marrow infiltration.
- Despite extensive disease, complete clinical response was achieved with first-line COP chemotherapy and rituximab.
- The patient remains in good condition with over 2 years of follow-up.
Implications:
- This case underscores the possibility of successful treatment even in rare, advanced presentations of follicular lymphoma.
- Early diagnosis and combination therapy including rituximab can lead to durable remissions.
- Further research into the mechanisms of leukemic transformation in FL may inform treatment strategies.