Diagnosis and management of rare paediatric Non-Hodgkin lymphoma

Xavier, Ana C.; Suzuki, Ritsuro; Attarbaschi, Andishe

doi:10.1016/j.beha.2023.101440

Cited by 8 publications

(4 citation statements)

References 115 publications

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“…Extranodal NK/T-cell lymphoma (ENKTL, previously ENKTL, nasal type) is a rare subtype of mature T/NK cell neoplasms in the 5th edition of the World Health Organization classification of hematolymphoid tumors in 2022 (WHO-HAEM5) [ 1 – 4 ]. ENKTL is characterized by universal extranodal involvement and is closely associated with invariable Epstein-Barr virus (EBV) infection [ 5 , 6 ].…”

Section: Introductionmentioning

confidence: 99%

“…Currently, there is a lack of established standard first-line treatment for ENKTL, particularly in children, despite the conduct of numerous prospective studies exploring novel drugs and showing improved patient outcomes [ 16 , 17 ]. Hence, there is a critical requirement to establish an effective frontline treatment approach for ENKTL [ 4 ]. RT combined with chemotherapy is the most widely used treatment for ENKTL [ 8 ].…”

Section: Introductionmentioning

confidence: 99%

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“Sandwich” protocol based on modified SMILE regimen for children with newly extranodal NK/T cell lymphoma, nasal type: a single-arm, single-center clinical study

Shen

Wan

et al. 2023

Ann Hematol

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Extranodal NK/T-cell lymphoma, nasal type (ENKTL), which is a rare form of mature T/NK cell lymphoma in children, currently lacks a standardized first-line treatment approach. However, a treatment protocol known as the “sandwich” regimen has been used in children newly diagnosed with ENKTL. This protocol combines the administration of methotrexate, ifosfamide, etoposide, pegaspargase, and dexamethasone (referred to as SMILE) with the addition of radiotherapy (RT). From September 2017 to December 2020, a total of five patients were included in the study, consisting of three males and two females. The median age of onset was 10.6 years (range, 9.8 to 14.0 years). Among the patients, four had nasal/nasopharyngeal disease at stage II, while one patient had extra nasal disease involving the skin at stage IV. The median EBV-DNA level in plasma was 1.68 × 103 copies/ml (range, 0.44 to 21.1 × 103copies/ml). All the patients had good overall response after 2 cycles of chemotherapy and radiotherapy, including 4 of the patients who had a complete response and 1 of the patients with partial remission. The patient with stage IV received allogeneic hematopoietic stem cell transplantation after the EBV-DNA level was elevated again during treatment. One patient in the low-risk group experienced grade 4 oral mucositis, while no other severe complications or treatment-related deaths were observed. The median follow-up period was 22 months (range, 5 to 57 months). All five patients successfully completed their treatment, with four patients achieving event-free survival, and one patient was lost to follow-up. The median OS time and EFS time was 33 months (range: 18–57 months) and 20 months (range: 5–47 months), respectively. The sandwich protocol has demonstrated a high response rate, good tolerance to chemotherapy, and no treatment-related fatalities. However, further confirmation is necessary through additional clinical studies involving larger sample sizes. Clinical trial registration number: Due to modified SMILE regimens with sandwiched radiotherapy yielded promising outcomes in children ENKTL, we have carried out a phase II multicenter clinical trial (ChiCTR220005954) for children ENKTL in China to further verify the efficacy and safety.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

“Sandwich” protocol based on modified SMILE regimen for children with newly extranodal NK/T cell lymphoma, nasal type: a single-arm, single-center clinical study

Shen

Wan

et al. 2023

Ann Hematol

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“…In addition, only 6 of 19 patients (32%) with complete resection had a purely follicular LBCL- IRF4+ , which, due to its clinical and biological closeness to pediatric-type follicular lymphoma, could have indicated a watchful waiting approach, but this was only done in 1 patient. 11 , 12 , 24 , 25 , 26 …”

mentioning

confidence: 99%

“…Such trials might also test watchful waiting for (in)completely resected, purely follicular LBCL- IRF4+ of the head-and-neck region. 12 , 24 , 25 , 26 , 27 …”

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confidence: 99%