Large B-cell lymphoma with IRF4 rearrangement: a special tonsillar lymphoma in children
Objective: To identify the clinicopathological features and prognosis of large B-cell lymphoma(LBCL) with interferon regulation factor 4 (IRF4) rearrangement. Methods: We retrospectively analyzed the medical records of four patients from Children's Hospital of Fudan University diagnosed with LB...
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Elsevier,
2019-12-01T00:00:00Z.
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| LEADER | 00000 am a22000003u 4500 | ||
|---|---|---|---|
| 001 | doaj_b86005a2bb524d4bb18f4e87c76eef11 | ||
| 042 | |a dc | ||
| 100 | 1 | 0 | |a Jie Man |e author |
| 700 | 1 | 0 | |a Yizhen Wang |e author |
| 700 | 1 | 0 | |a Xiaowen Zhai |e author |
| 700 | 1 | 0 | |a Lian Chen |e author |
| 700 | 1 | 0 | |a Yangyang Ma |e author |
| 700 | 1 | 0 | |a Hongsheng Wang |e author |
| 245 | 0 | 0 | |a Large B-cell lymphoma with IRF4 rearrangement: a special tonsillar lymphoma in children |
| 260 | |b Elsevier, |c 2019-12-01T00:00:00Z. | ||
| 500 | |a 2468-1245 | ||
| 500 | |a 10.1016/j.phoj.2019.12.001 | ||
| 520 | |a Objective: To identify the clinicopathological features and prognosis of large B-cell lymphoma(LBCL) with interferon regulation factor 4 (IRF4) rearrangement. Methods: We retrospectively analyzed the medical records of four patients from Children's Hospital of Fudan University diagnosed with LBCL with IRF4 rearrangement during Sep. 2014 to Oct. 2018, and performed a literature review. Results: Four patients had an average age of 5.7 years. The median duration from presentation to final diagnosis was 25.7 days. All 4 patients were admitted to the hospital due to severe snoring with no causes. The patients showed enlarged lymph nodes in the neck without tenderness. All 4 cases presented as localized disease (St-jude stage II). Lactate dehydrogenase (LDH) and serum albumin were all in the normal range. Morphology showed that normal lymphoid follicles were diffusely invaded by consistent, medium-sized large cells with obvious nucleoli. CD20, CD79α, PAX5, CD10 and BCL6 were diffusely positive. Neoplastic cells were also strongly positive for MUM1 and Ki-67. IRF4 gene rearrangement was detected as positive by IRF4 dual-color break-apart probe. Four patients underwent routine chemotherapy post bilateral tonsillectomy operation. Three achieved complete remission (CR) while the newly diagnosed one is during the second course of chemotherapy. Conclusions: Tonsillar LBCL with IRF4 rearrangement in children presents distinct clinicopathologic and molecular genetic features, and it usually has a favorable outcome. Keywords: Large B-cell lymphoma, IRF4 rearrangement, Child, Tonsil, Prognosis | ||
| 546 | |a EN | ||
| 690 | |a Pediatrics | ||
| 690 | |a RJ1-570 | ||
| 655 | 7 | |a article |2 local | |
| 786 | 0 | |n Pediatric Hematology Oncology Journal, Vol 4, Iss 4, Pp 77-81 (2019) | |
| 787 | 0 | |n http://www.sciencedirect.com/science/article/pii/S2468124519302578 | |
| 787 | 0 | |n https://doaj.org/toc/2468-1245 | |
| 856 | 4 | 1 | |u https://doaj.org/article/b86005a2bb524d4bb18f4e87c76eef11 |z Connect to this object online. |