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[Angioimmunoblastic T-cell lymphoma presenting with rapidly increasing biclonal gammopathy]
T Tasaka1, Y Matsuhashi, E Uehara
1Department of Medicine, Kagawa Prefectural, Central Hospital.
Insights
This study reports a rare case of angioimmunoblastic T-cell lymphoma presenting with biclonal gammopathy. The findings highlight a complex interplay between lymphoma, immune dysregulation, and protein abnormalities.
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- Angioimmunoblastic T-cell lymphoma (AITL) is an aggressive non-Hodgkin lymphoma characterized by systemic symptoms and lymphadenopathy.
- Gammopathy, particularly monoclonal gammopathy, can occur in AITL, but biclonal gammopathy is exceptionally rare.
Observation:
- A 59-year-old male presented with fever, dysphagia, a lingual tumor, and enlarged cervical and inguinal lymph nodes.
- Lymph node biopsy revealed effacement of normal architecture by atypical lymphocytes and prominent high endothelial venules.
- Genetic analysis confirmed T-cell receptor beta gene rearrangement, supporting a T-cell origin.
Findings:
- The patient was diagnosed with angioimmunoblastic T-cell lymphoma.
- Serial protein analysis revealed rapidly increasing M-protein with immunofixation electrophoresis confirming biclonal gammopathy (IgG kappa and IgM lambda).
- This represents a rare co-occurrence of biclonal gammopathy in AITL.
Implications:
- The case underscores the importance of comprehensive protein analysis in AITL patients to detect rare complications like biclonal gammopathy.
- Further research is warranted to elucidate the mechanisms linking AITL, biclonal gammopathy, and potential viral associations (HHV-6, HHV-8, EBV).
- Understanding these associations may lead to improved diagnostic and therapeutic strategies for AITL.
Abstract:
A 59-year-old man was admitted to our hospital with high fever and dysphagia. Physical examination revealed a lingual tumor and swelling of the cervical and inguinal lymph nodes. A lymph node biopsy specimen revealed that the normal nodal architecture had been obliterated by infiltration of small, intermediate, and large pale lymphocytes. Arborous branching of high endothelial venules was also observed. Southern blotting analysis showed rearrangement of the T-cell receptor beta gene. A diagnosis of angioimmunoblastic T-cell lymphoma was made. Serial serum protein fractionation analysis showed rapidly increasing M-protein. Immunofixation electrophoresis indicated specific bands for IgG kappa and IgM lambda, confirming the presence of biclonal gammopathy. Biclonal gammopathy has rarely been reported in patients with angioimmunoblastic T-cell lymphoma. The relationship between the occurrence of biclonal gammopathy and the possible role of HHV-6, HHV-8 and EBV is discussed.
