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

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

TLDR

  • The study found that pediatric ALCL often presents with advanced-stage disease, but early treatment and recognition of skin lesions improve outcomes

Abstract

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. 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. 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. 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.

Overview

  • The study investigated the clinical characteristics of children diagnosed with systemic anaplastic large cell lymphomas (ALCLs) at Valme University Hospital.
  • The research aimed to identify the demographic, clinical, and treatment characteristics of pediatric patients with systemic ALCL.
  • The study intended to highlight the importance of prompt recognition and early intervention in managing pediatric ALCL.

Comparative Analysis & Findings

  • The study found that the mean age at diagnosis was 8.5 years, with a male predominance (78.6%)
  • The most common presentation was cutaneous (35.7%), characterized by rapidly growing subcutaneous nodules
  • All cases had anaplastic lymphoma kinase (ALK) rearrangement, and anthracycline-based chemotherapy resulted in 100% 5- and 10-year overall survival rates

Implications and Future Directions

  • The study highlights the importance of vigilance and early intervention in managing pediatric ALCL
  • Prompt recognition of skin lesions is vital to expedite diagnosis and treatment initiation, ultimately improving patient outcomes
  • Future research could focus on identifying additional risk factors and developing more effective treatment strategies for pediatric ALCL
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