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[Angioimmunoblastic T-cell lymphoma with hyperplastic germinal centers].
K Kawakami1, Y Watanabe, F Momma
1Division of Hematology, JA Suzuka Hospital.
[Rinsho Ketsueki] the Japanese Journal of Clinical Hematology
|January 26, 2002
Summary
This study details a rare case of angioimmunoblastic T-cell lymphoma (AITL) presenting with unusual hyperplastic germinal centers. The patient achieved remission with chemotherapy, highlighting AITL
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- Angioimmunoblastic T-cell lymphoma (AITL) is an aggressive non-Hodgkin lymphoma.
- AITL typically presents with systemic symptoms and lymphadenopathy.
- Histopathological features are crucial for AITL diagnosis.
Observation:
- A 48-year-old male presented with characteristic AITL symptoms including fever, weight loss, rash, lymphadenopathy, Coombs positivity, and polyclonal hypergammaglobulinemia.
- Histopathology revealed preserved nodal architecture and hyperplastic germinal centers, an atypical finding for AITL.
- Increased intrafollicular tingible-body macrophages and interfollicular proliferation of UCHL-1+ immunoblasts were noted.
Findings:
- The T-cell receptor gene (C beta 1) demonstrated clonal rearrangement, supporting a neoplastic T-cell process.
- The diagnosis of AITL with hyperplastic germinal centers was established despite the unusual germinal center morphology.
- The patient responded well to six courses of CHOP chemotherapy.
Implications:
- This case expands the histopathological spectrum of angioimmunoblastic T-cell lymphoma.
- It underscores the importance of integrating clinical, immunophenotypic, and molecular findings for accurate AITL diagnosis.
- Successful treatment with CHOP suggests its efficacy even in atypical AITL presentations.