Clinical Characteristics and Outcomes of Pediatric Systemic Anaplastic Large Cell Lymphoma.

IF 1.2 4区医学 Q3 DERMATOLOGY

Pediatric Dermatology Pub Date : 2025-05-01 Epub Date: 2025-01-12 DOI:10.1111/pde.15850

Juan-Manuel Morón-Ocaña, Pablo Monsalve-Sosa, María-Luisa Martínez-Barranca, Isabel-María Coronel-Pérez, Amalia Pérez-Gil

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引用次数: 0

Abstract

Background/objectives: Anaplastic large cell lymphomas (ALCLs) present unique challenges due to their clinical and genetic heterogeneity. This study investigated the clinical characteristics of children diagnosed with systemic ALCL.

Methods: Retrospective data from 14 pediatric patients diagnosed with systemic ALCL at Valme University Hospital were studied. Demographic, clinical, and treatment data were collected and statistically analyzed.

Results: The mean age at diagnosis was 8.5 years, with a male predominance (78.6%). Cutaneous presentation occurred in 35.7% of cases, with characteristic rapidly growing subcutaneous nodules. B symptoms were present in 57.1% of patients, while 100% exhibited nodal involvement. Visceral and bone marrow involvement was observed in 71.4% and 7.1% of patients, respectively. Central nervous system (CNS) involvement was absent. Anaplastic lymphoma kinase (ALK) rearrangement was positive in all cases. Anthracycline-based chemotherapy resulted in 100% 5- and 10-year overall survival rates.

Conclusions: Systemic ALCL in children often presents with advanced-stage disease, with cutaneous involvement in a significant proportion of cases. Prompt recognition of skin lesions is vital to expedite diagnosis and treatment initiation, ultimately improving patient outcomes. This study underscores the importance of vigilance and early intervention in managing pediatric ALCL.

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小儿全身性间变性大细胞淋巴瘤的临床特点及预后。

背景/目的：间变性大细胞淋巴瘤（alcl）由于其临床和遗传异质性而面临独特的挑战。本研究探讨全身性ALCL患儿的临床特点。方法：回顾性分析Valme大学医院14例全身性ALCL患儿的资料。收集人口学、临床和治疗数据并进行统计分析。结果：平均诊断年龄8.5岁，男性占78.6%。35.7%的病例出现皮肤表现，以快速生长的皮下结节为特征。57.1%的患者出现B型症状，而100%的患者表现为淋巴结受累。内脏和骨髓受累分别为71.4%和7.1%。中枢神经系统（CNS）未受累。间变性淋巴瘤激酶（ALK）重排阳性。蒽环类化疗的5年和10年总生存率为100%。结论：儿童全身性ALCL通常表现为晚期疾病，在很大比例的病例中伴有皮肤受累。及时识别皮肤病变对于加快诊断和治疗开始至关重要，最终改善患者的预后。本研究强调了警惕和早期干预在处理儿童ALCL中的重要性。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Pediatric Dermatology 医学-皮肤病学

CiteScore

3.20

自引率

6.70%

发文量

269

审稿时长

1 months

期刊介绍： Pediatric Dermatology answers the need for new ideas and strategies for today''s pediatrician or dermatologist. As a teaching vehicle, the Journal is still unsurpassed and it will continue to present the latest on topics such as hemangiomas, atopic dermatitis, rare and unusual presentations of childhood diseases, neonatal medicine, and therapeutic advances. As important progress is made in any area involving infants and children, Pediatric Dermatology is there to publish the findings.