Çalışkan Kamış Şule, Yağcı Begül
Department of Pediatric Hematology and Oncology, University of Health Sciences, Adana Faculty of Medicine, Adana City Education and Research Hospital, Adana, Turkey.
Ann Med Surg (Lond). 2025 Jun 25;87(8):5209-5212. doi: 10.1097/MS9.0000000000003506. eCollection 2025 Aug.
T-lymphoblastic lymphoma (T-LBL) is a rare and aggressive subtype of precursor T-cell lymphomas, often presenting with lymph node, bone marrow, and mediastinal involvement. Cutaneous involvement is uncommon but associated with a poor prognosis.
We report the case of a 17-year-old female with relapsed T-LBL presenting with bilateral breast masses, confirmed by biopsy and immunohistochemistry. Initial treatment with a BFM ALL chemotherapy protocol resulted in partial response, but disease recurrence with cutaneous and cervical lymph node involvement necessitated further treatment with the ALLIC BFM 2016 Relapse protocol. The disease course was complicated by avascular necrosis requiring hip arthroplasty, and follow-up PET/CT scans revealed further extranodal involvement, including the skin.
Extranodal involvement in peripheral T-cell lymphomas (PTCL), such as T-LBL, poses significant challenges to clinical management due to its association with aggressive disease and poor prognosis. Standard CHOP regimens often result in short-term remission with high relapse rates. Emerging therapies, including HDAC inhibitors, proteasome inhibitors, and immunotherapies, show promise but require further study to address tumor heterogeneity and optimize efficacy.
This case underscores the critical role of early diagnosis and effective multidisciplinary management in relapsed T-LBL with skin involvement. Further research is needed to develop personalized therapeutic strategies to improve outcomes in this rare and aggressive lymphoma subtype.
T淋巴细胞母细胞淋巴瘤(T-LBL)是一种罕见且侵袭性强的前体T细胞淋巴瘤亚型,常表现为淋巴结、骨髓和纵隔受累。皮肤受累并不常见,但与预后不良相关。
我们报告了一例17岁复发T-LBL女性患者,表现为双侧乳腺肿块,经活检和免疫组化确诊。最初采用BFM ALL化疗方案治疗取得部分缓解,但疾病复发并伴有皮肤和颈部淋巴结受累,需要采用ALLIC BFM 2016复发方案进一步治疗。疾病过程因无血管性坏死而复杂化,需要进行髋关节置换术,后续的PET/CT扫描显示有更多结外受累,包括皮肤。
外周T细胞淋巴瘤(PTCL)如T-LBL的结外受累,因其与侵袭性疾病和预后不良相关,给临床管理带来了重大挑战。标准的CHOP方案通常只能带来短期缓解且复发率高。包括组蛋白去乙酰化酶抑制剂、蛋白酶体抑制剂和免疫疗法在内的新兴疗法显示出前景,但需要进一步研究以应对肿瘤异质性并优化疗效。
该病例强调了早期诊断和有效的多学科管理在复发且伴有皮肤受累的T-LBL中的关键作用。需要进一步研究以制定个性化治疗策略,改善这种罕见且侵袭性淋巴瘤亚型的治疗结果。