Clinical Features and Treatment Outcomes of Children with Anaplastic Large Cell Lymphoma

Objective: To describe clinical features and treatment options in pediatric patients with ALCL (Anaplastic large cell lymphoma) and their outcome over a span of 10 years. Study Design: A retrospective-observational study. Place and duration of study: Shaukat Khanum Memorial Cancer Hospital and Research Centre, Lahore, from January 2005 to December 2015. Methodology: Medical records of pediatric patients with anaplastic large cell lymphoma was retrospectively collected after IRB approval. Data was reviewed for patients confirmed on histopathology and age less than 20 years at the time of diagnosis to see clinical features and treatment outcomes. Descriptive statistics were applied. Results: A total of 40 children, 27 males (67.5%) and 13 females (32.5%) with ALCL (CD30 +), were reviewed. B symptoms were present in 32 (80%) patients, nodal involvement in 39 (97.5%), and mediastinum involvement was present in 8 (20%) patients. Visceral (lung, liver, spleen) and cutaneous involvement was seen in 16 (40%) and 6 (15%) patients, respectively. ALK was positive in 19 patients (48%) and Bone marrow was involved in 3 patients (7.5%). Stage III was seen in 29 (72.5%). All patients were treated on ALCL 99 protocol. Five-year EFS (event-free survival) and OS (overall survival) was 30 and 60%, respectively. There were 7 relapses, 2 progressive disease, 16 death and 3 refusal for treatment. Conclusion: This analysis shows poor outcomes in pediatric ALCL. The most common cause of mortality was hematological toxicity and febrile neutropenia associated with it. Supportive care needs to be improved.