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Primary cutaneous marginal center lymphoma - complete remission induced by interferon alpha2a
U Wollina1, S Hahnfeld, H Kosmehl
1Department of Dermatology, Friedrich-Schiller-University Jena, Germany. uwol@derma.uni-jena.de
Insights
This case study shows interferon alpha2a effectively treated a rare form of marginal zone lymphoma (MZL) affecting the skin. The patient achieved complete remission with minimal side effects, highlighting a potential therapeutic option for this B-cell lymphoma.
Area of Science:
- Hematology
- Oncology
- Dermatology
Background:
- Marginal zone lymphoma (MZL) is a specific subtype of B-cell lymphoma.
- Primary cutaneous MZL presents unique diagnostic and therapeutic challenges.
Observation:
- A 53-year-old woman presented with disseminated primary cutaneous MZL and secondary lymph node involvement.
- Tumor cells exhibited a CD20/CD79a/kappa/lambda+/bcl-2 positive phenotype with low Ki-67 proliferation.
- No systemic or bone marrow involvement was detected, classifying the disease as Stage IVa MZL with plasmacytic differentiation.
Findings:
- Treatment with subcutaneous interferon alpha2a (9x10^6 U thrice weekly for 1 year) led to complete resolution of skin lesions.
- No lymph node or extracutaneous recurrence was observed during the treatment period.
- The patient maintained complete remission with only Grade I WHO side effects and a Karnofsky index of 90%.
Implications:
- Prolonged interferon alpha monotherapy demonstrates efficacy in managing primary cutaneous MZL.
- This treatment approach may induce complete remission and control disease progression in MZL patients.
- Interferon alpha represents a viable therapeutic strategy for specific B-cell lymphomas, including cutaneous MZL.
Abstract:
Marginal zone lymphoma (MZL) is a distinct entity among B-cell lymphomas. We report on a 53-year-old woman who developed disseminated primary cutaneous MZL with secondary lymph node involvement and perinodular spreading. The tumor cell phenotype was characterized as CD20/CD79a/kappa/lambda+/ bcl-2-positive, CD3/5/15/39/bcl-1-negative. Ki-67 was expressed by 20-35% of tumor cells. There was no evidence of systemic (including bone marrow) involvement. The diagnosis of MZL with plasmacellular differentiation (Stage IVa) was made. The patient was treated with interferon alpha2a injected s.c. at 9x10(6) U 3 days a week for 1 year. During this time the skin lesions completely disappeared. No evidence of lymph node or extracutaneous disease was found. The patient remains in complete remission. Side effects were only of grade I (WHO); the Karnovsky index was 90%. As shown for other types of primary cutaneous B-cell lymphoma, prolonged interferon alpha monotherapy may be effective in controlling the disease and/or inducing complete remission in MZL.