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Table 1.

Pathological and clinical features of 20 LBCL cases with IRF4 rearrangement

Case Age (y), gender Biopsy site Growth pattern Immunohistochemistry FISH COO NanoString (Lymph2Cx) Stage* Treatment Outcome, follow-up
CD10 BCL6 BCL2 IRF4/MUM1 IRF4 IGH
D1 5, F LN Follicular and diffuse + + + +
D2 14, M LN Follicular + + + R GCB Surgical excision
D15 5, F Tonsil Diffuse + + + R R GCB II CT-P CR, 49 mo
D16 14, F Tonsil Diffuse + + R II CT-P CR, 99 mo
D17 21, M Liver Diffuse + + + R ABC I-E CT-A CR, 10 mo
D20 17, M Ileum Diffuse + + + + N R UNC II CT-P CR, 72 mo
D21 8, M Tonsil Follicular + + + + R I CT-P CR, 36 mo
D23 21, F Inguinal LN Diffuse + + R R GCB IV-A CT-A CR, 40 mo
D31 12, F Cervical LN Diffuse + + + N R UNC
D32 7, M Tonsil Follicular + + + + R GCB I CT-P CR, 22 mo
D35 6, F Cervical LN Diffuse + + + R GCB I CT-P CR, 63 mo
D46 11, M Tonsil Follicular and diffuse + + + R R GCB Surgical excision
D47 22, M Tonsil Follicular and diffuse + + + R R I CT-A CR, 24 mo
D48 18, F Tonsil Follicular and diffuse + + R UNC I CT-A CR, 29 mo
D50 17, F Cervical LN Follicular + + R R Surgical excision
D51 10, F Tonsil Follicular and diffuse + + + R GCB III CT-P CR, 14 mo
D54 18, F Cervical LN Diffuse + + + + R GCB III CT-P CR, 14 mo
D62 17, M Cervical LN Follicular + + R GCB II CT-P CR, 45 mo
D63 14, F Larynx Diffuse + + + + R GCB I CT-P CR, 18 mo
D69 15, M Intestine Follicular and diffuse + + + R CT-P CR, 10 mo

CR, complete response; CT-A, chemotherapy with adult schema protocol (R-CHOP/ESHAP); CT-P, chemotherapy with pediatric schema protocol; F, female; LN, lymph node; M, male; N, normal; R, rearrangement; UNC, intermediate/unclassified.

*

Stage was established according St. Jude/International Pediatric NHL Staging System or Ann Arbor staging system for pediatric and adult patients, respectively.

Patients who had a relapse/progression and needed rescue treatment.