A case of angioimmunoblastic T-cell lymphoma coexisting with castleman disease

Angioimmunoblastic T-cell lymphoma (AITL) is a rare, mature T-cell lymphoma with diagnostic challenges. Its coexistence with Castleman disease (CD) is exceptionally uncommon and complicates diagnosis and management. We report a 67-year-old male with bilateral cervical lymphadenopathy. Imaging showed...

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Veröffentlicht in:Human Pathology Reports Jg. 42; S. 300798
Hauptverfasser: Zhan, Lianshan, Wang, Long, Wu, Hongyan, Chen, Zhongjiao, Wang, Lina
Format: Journal Article
Sprache:Englisch
Veröffentlicht: Elsevier Inc 01.11.2025
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Abstract Angioimmunoblastic T-cell lymphoma (AITL) is a rare, mature T-cell lymphoma with diagnostic challenges. Its coexistence with Castleman disease (CD) is exceptionally uncommon and complicates diagnosis and management. We report a 67-year-old male with bilateral cervical lymphadenopathy. Imaging showed enlarged lymph nodes with prominent vascularity and a “rim sign.” Initial pathology suggested CD, with preserved lymph node architecture, atrophic germinal centers, and “lollipop” structures. Further evaluation, however, revealed diffuse proliferation of atypical T-cells. Immunohistochemistry showed atypical cells expressing follicular helper T-cell markers (CD10, PD1, BCL6). Molecular analysis confirmed monoclonal TCRγ gene rearrangement, and flow cytometry identified an abnormal CD4 + CD7 − T-cell clone. These findings established the diagnosis of AITL coexisting with CD. This case highlights the high diagnostic hurdles of coexisting AITL and CD, emphasizing the critical need for comprehensive morphological, immunophenotypic, and molecular analyses for accurate diagnosis and optimal patient management.
AbstractList AbstractBackgroundAngioimmunoblastic T-cell lymphoma (AITL) is a rare, mature T-cell lymphoma with diagnostic challenges. Its coexistence with Castleman disease (CD) is exceptionally uncommon and complicates diagnosis and management. Case presentationWe report a 67-year-old male with bilateral cervical lymphadenopathy. Imaging showed enlarged lymph nodes with prominent vascularity and a “rim sign.” Initial pathology suggested CD, with preserved lymph node architecture, atrophic germinal centers, and “lollipop” structures. Further evaluation, however, revealed diffuse proliferation of atypical T-cells. Diagnostic findingsImmunohistochemistry showed atypical cells expressing follicular helper T-cell markers (CD10, PD1, BCL6). Molecular analysis confirmed monoclonal TCRγ gene rearrangement, and flow cytometry identified an abnormal CD4 + CD7 − T-cell clone. These findings established the diagnosis of AITL coexisting with CD. ConclusionThis case highlights the high diagnostic hurdles of coexisting AITL and CD, emphasizing the critical need for comprehensive morphological, immunophenotypic, and molecular analyses for accurate diagnosis and optimal patient management.
Angioimmunoblastic T-cell lymphoma (AITL) is a rare, mature T-cell lymphoma with diagnostic challenges. Its coexistence with Castleman disease (CD) is exceptionally uncommon and complicates diagnosis and management. We report a 67-year-old male with bilateral cervical lymphadenopathy. Imaging showed enlarged lymph nodes with prominent vascularity and a “rim sign.” Initial pathology suggested CD, with preserved lymph node architecture, atrophic germinal centers, and “lollipop” structures. Further evaluation, however, revealed diffuse proliferation of atypical T-cells. Immunohistochemistry showed atypical cells expressing follicular helper T-cell markers (CD10, PD1, BCL6). Molecular analysis confirmed monoclonal TCRγ gene rearrangement, and flow cytometry identified an abnormal CD4 + CD7 − T-cell clone. These findings established the diagnosis of AITL coexisting with CD. This case highlights the high diagnostic hurdles of coexisting AITL and CD, emphasizing the critical need for comprehensive morphological, immunophenotypic, and molecular analyses for accurate diagnosis and optimal patient management.
Background: Angioimmunoblastic T-cell lymphoma (AITL) is a rare, mature T-cell lymphoma with diagnostic challenges. Its coexistence with Castleman disease (CD) is exceptionally uncommon and complicates diagnosis and management. Case presentation: We report a 67-year-old male with bilateral cervical lymphadenopathy. Imaging showed enlarged lymph nodes with prominent vascularity and a “rim sign.” Initial pathology suggested CD, with preserved lymph node architecture, atrophic germinal centers, and “lollipop” structures. Further evaluation, however, revealed diffuse proliferation of atypical T-cells. Diagnostic findings: Immunohistochemistry showed atypical cells expressing follicular helper T-cell markers (CD10, PD1, BCL6). Molecular analysis confirmed monoclonal TCRγ gene rearrangement, and flow cytometry identified an abnormal CD4 + CD7 − T-cell clone. These findings established the diagnosis of AITL coexisting with CD. Conclusion: This case highlights the high diagnostic hurdles of coexisting AITL and CD, emphasizing the critical need for comprehensive morphological, immunophenotypic, and molecular analyses for accurate diagnosis and optimal patient management.
ArticleNumber 300798
Author Wang, Long
Chen, Zhongjiao
Wang, Lina
Zhan, Lianshan
Wu, Hongyan
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Cites_doi 10.1097/MD.0000000000018650
10.3960/jslrt.21036
10.1007/s00277-024-05817-6
10.1111/bjh.19171
10.1186/s40644-018-0163-7
10.1038/s41375-022-01620-2
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Keywords PTCL-NOS
Rim sign
CD
CT
TCR
FDC
Castleman disease (CD)
TFH
HEV
Angioimmunoblastic T-cell lymphoma (AITL)
AITL
T follicular helper (TFH)
High Endothelial Venules
Angioimmunoblastic T-cell Lymphoma
T-cell Receptor
T follicular helper
Peripheral T-cell Lymphoma, Not Otherwise Specified
Computed Tomography
Follicular Dendritic Cell
Castleman Disease
Language English
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Snippet Angioimmunoblastic T-cell lymphoma (AITL) is a rare, mature T-cell lymphoma with diagnostic challenges. Its coexistence with Castleman disease (CD) is...
AbstractBackgroundAngioimmunoblastic T-cell lymphoma (AITL) is a rare, mature T-cell lymphoma with diagnostic challenges. Its coexistence with Castleman...
Background: Angioimmunoblastic T-cell lymphoma (AITL) is a rare, mature T-cell lymphoma with diagnostic challenges. Its coexistence with Castleman disease (CD)...
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SubjectTerms Angioimmunoblastic T-cell lymphoma (AITL)
Castleman disease (CD)
Pathology
Rim sign
T follicular helper (TFH)
Title A case of angioimmunoblastic T-cell lymphoma coexisting with castleman disease
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