Histologic evolution of angioimmunoblastic T-cell lymphoma in consecutive biopsies: clinical correlation and insights into natural history and disease progression.
Angioimmunoblastic T-cell lymphoma (AITL) is an uncommon, but aggressive nodal peripheral T-cell lymphoma. Little is known of its biology and its natural history has been poorly studied. We report the first comprehensive study on the natural history/histologic progression of AITL by reviewing consecutive biopsies in 31 cases. Immunostaining for CD3, CD20, CD10 and CD21, CD23, CNA-42, CD4, CD8, and Ki 67, in situ hybridization for Epstein-Barr virus (EBV)-encoded RNA and polymerase chain reaction for T-clonality and B-clonality were performed. Histologic progression from AITL with limited nodal involvement and hyperplastic follicles (pattern I) to typical AITL with or without regressed follicles (patterns II and III) was observed in 7 cases, one of which relapsed subsequently as pattern I. Thirteen cases showed typical AITL at presentation and follow-up. Eleven cases where polymerase chain reaction results for T-cell receptor-gamma gene rearrangement were directly compared showed an identical band-size in the initial and follow-up biopsies. Seven cases (23%) developed EBV-associated B-cell lymphomas [5 diffuse large B-cell lymphoma (DLBCL) and 2 classic Hodgkin lymphoma]. In 4 cases, a dominant B-cell clone was observed in biopsies lacking evidence of DLBCL. A single case was complicated by EBV-negative DLBCL, whereas another with large cell transformation had a T-cell phenotype. In conclusion, AITL represents a clonal T-cell proliferation with a stable T-cell clone throughout the disease. Partial nodal involvement with hyperplastic follicles is seen in early AITL and at relapse. When "morphologic high-grade transformation" occurs, it is usually due to a secondary (often EBV-associated) B-cell lymphoma, rather than a T-cell neoplasm.
Attygalle
Ayoma Deepthi
AD
Department of Histopathology, Royal Marsden Hospital, London, United Kingdom.
Kyriakou
Charalampia
C
Dupuis
Jehan
J
Grogg
Karen Lynne
KL
Diss
Timothy Charles
TC
Wotherspoon
Andrew Charles
AC
Chuang
Shih Sung
SS
Cabeçadas
José
J
Isaacson
Peter Gershon
PG
Du
Ming-Qing
MQ
Gaulard
Philippe
P
Dogan
Ahmet
A
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000378:metabolism; D001706:Biopsy; D002999:Clone Cells / Q000378:metabolism / Q000473:pathology; D018450:Disease Progression; D018572:Disease-Free Survival; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000378:metabolism / Q000473:pathology; D005260:Female; D015329:Gene Rearrangement, T-Lymphocyte; D004854:Herpesvirus 4, Human / Q000302:isolation & purification; D006801:Humans; D017403:In Situ Hybridization; D008198:Lymph Nodes / Q000378:metabolism / Q000473:pathology*; D016411:Lymphoma, T-Cell, Peripheral / Q000188:drug therapy / Q000209:etiology / Q000378:metabolism / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D012367:RNA, Viral / Q000032:analysis; D013601:T-Lymphocytes / Q000378:metabolism / Q000473:pathology*; D016896:Treatment Outcome
D014408:Biomarkers, Tumor; D012367:RNA, Viral