Background Anaplastic large cell lymphoma (ALCL) in children is usually ALK positive and is characterised by advanced disease at presentation with a high incidence of extra nodal involvement. We present the clinical profile, treatment and outcome of a small cohort of children with ALCL treated at our centre between January 2004 and June 2013.
Results 19 children, 14 boys and 5 girls, aged 18 months to 14 years, with ALCL. 16/19 had lymphadenopathy and 14/19 had fever. Thirteen had high risk, 5 had standard risk and one had low risk disease. 16/19 ALK positive. 17/19 were treated according to the NHL-BFM 90 protocol. Fourteen completed treatment. Three died during treatment; 2 due to infection and 1 had progressive disease. All 3 had high risk disease and were ALK positive. All thethree children with ALK negative disease relapsed; one died on relapse treatment, another was lost to follow up a year after completion of relapse therapy and the third with primary cutaneous disease, is alive and well. Of the 11 ALK positive children who completed treatment, only one relapsed and died. The remaining 10 are alive and well with a mean follow up of 17 months (range 8–93 months).
Conclusion The majority of our patients are ALK positive and have high risk disease at presentation. 55% of children with high risk disease are alive and well after a mean follow up of 17 months. The overall survival was 65% and event free survival 58% at the time of this analysis.