Detection of immunoglobulin and T-cell receptor gene rearrangements in angioimmunoblastic T-cell lymphoma

Objective: To assess the value of immunoglobulin and T-cell receptor gene rearrangements in the diagnosis and differential diagnosis of angioimmunoblastic T-cell lymphoma. Methods: We selected 55 cases of angioimmunoblastic T-cell lymphoma confirmed by histopathology and 15 cases of reactive lymph node hyperplasia. Using the IdentiClone gene rearrangement detection kit, BIOMED-2 primer system, and GeneScanning analysis, we tested for immunoglobulin and T-cell receptor gene rearrangements. Results: Among all 55 angioimmunoblastic T-cell lymphoma cases, 1 (2%) displayed the first type of angioimmunoblastic T-cell lymphoma, which has an intact lymphoid follicle structure. Five cases (9%) displayed the second type, which has an intact segmental lymphatic follicular structure. Forty-nine cases (89%) displayed the third type, which is characterized by a complete obliteration of the lymphatic follicular structure. Fifty-two cases (95%) had tumor cells that were positive for CD3, 50 cases (91%) were positive for CD4, 33 cases (60%) were positive for Bcl-6, 20 cases (36%) were positive for CD10, 44 cases (80%) were positive for CXCL13 to different degrees, and 53 cases (96%) showed a strong positive expression of CD21. Ki67 expression intensity was 30-80% in tumor T cells. Clonal gene rearrangements were identified in 48 of the 55 angioimmunoblastic T-cell lymphoma cases (87%), of which 30 (55%) displayed IG gene rearrangements, including IGHA (7 cases; 13%), IGHB (6 cases; 11%), IGHC (2 cases; 4%), IGKA (22 cases; 40%), IGKB (6 cases; 11%), and IGL (20 cases; 36%). TCR gene rearrangements were observed in 32 cases (58%), including TCRBA (6 cases; 11%), TCRBB (5 cases; 9%), TCRBC (10 cases; 18%), TCRD (7 cases; 13%), TCRGA (22 cases; 40%), and TCRGB (16 cases; 29%). IG and TCR gene rearrangements were concurrently observed in 14 cases (25%). Immunoglobulin or TCR clonal gene rearrangements were not detected in the 15 cases of reactive hyperplasia. Conclusions: Angioimmunoblastic T-cell lymphomas may be positive for immunoglobulin or T-cell receptor clone gene rearrangements or may express double rearrangements. The assessment of clonal gene rearrangements is valuable for the diagnosis and differential diagnosis of angioimmunoblastic T-cell lymphoma.