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Angioimmunoblastic
T-cell lymphoma
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Definition
-A subtype of peripheral T-cell lymphoma
-Systemic disease
-Polymorphous infiltrate involving LN
-Prominent proliferation of high endothelial venules
-Prominent proliferation of follicular dendritic cells
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Synonyms
Lukes-Collins: immunoblastic lymphadenopathy
Working Formulation: various categories
diffuse mixed small and large cell,
diffuse large cell,
immunoblastic,
atypical hyperplasia
Kiel: AILD-Type (lymphogranulomatosis X) T-cell lymphoma
REAL: angioimmunoblastic T-cell lymphoma
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Epidemiology
-Middle aged and elderly
-M=W
-15-20% of total PTCL
-1-2% of NHL
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Sites of involvement
-Generalized peripheral LNs
-Hepatosplenomegaly
-Skin rash
-BM involvement is common
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Clinical features
-Presents with advanced stage
-Systemic symptoms:
Skin rash, pruritus
Edema, pleural effusion, arthritis, ascites
-Association with drug hypersensitivity reactions (early series)
-Lab: polyclonal hypergammaglobulinemia,
immune complexes, cold agglutinins, hemolytic anemia,
rheumatoid factor, anti-smooth muscle Ab
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Etiology
-Immunodeficiency appears to be secondary to AITCL,
rather than preceding it
-EBV(75% of cases) in B-cells
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Precursor lesions of AITCL
-Angioimmunoblastic lymphadenopathy
(previous thinking)
-de novo PTCL(current thinking)
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Histopathology
-LN architecture: partially –completely effaced
-Regressed follicles
-Paracotex: polymorphous infiltrates
Lymhoma cells: medium to large, pale cytoplasm, distinct membrane,
minimal atypia
Others: small lymphocytes, plasma cells, eos, histiocytes, follicular
dendritic cells, rare HRS-like cells
-Infiltrate bridges capsule; distended cortical sinus; vascular proliferation
Distended cortical sinus
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Angioimmunoblastic T-Cell Lymphoma
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Histopathology(continued)
A-Hyperplastic germinal center
B-Large basophilic B blasts
C-RS-like cells
A B C
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Histopathology(continued)
-A: Arborizing blood vessels
-B: Dentric cells abut and extend from venues(CD21)
-C: medium-sized lymphoytes with clear cytoplasm/distinct membrane
-D: Double staining: EBER/CD20
A B
C
D
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Grading
Generally not performed
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Immunophenotype
-Positive for CD4 (predominant), CD3, CD2, CD5
-Admixed with many reactive CD8+ cells
-Also positive for TFH cell markers: CD10, CXCL13,
PD-1 (60-100% of cases)
-Polyclonal plasma cells
-CD21, CD21: conspicuous dendritic cells
Possible normal counterpart:
-CD4+ Follicular helper T cells (TFH) that are
activated by EBV-pos B cells
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Histopathology (continued)
CD3 CD20
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Genetics
TCR gene rearrangement: 75-90%
Ig gene rearrangement: 30% (in expanded EBV-pos B cells)
Cytogenetics: trisomy 3, trisomy 5, additional X
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Prognosis and predictive factors
-Aggressive
-Median survival: < 3 years
-Often succumb to infections
-Some patients may develop secondary EBV-pos DLBCL