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Anaplastic large cell lymphoma

A CD30- positive primary cutaneous T-cell lymphoma.

ALCL is a form of non-Hodgkin lymphoma accounting for 10% to 15% of all childhood lymphomas, with roughly 90% of pediatric cases containing a chromosomal translocation that may include ALK.

Has a high grade histology, and only 25% of lesions regress spontaneously.

Because its histologic appearance and frequent recurrence patients may be subjected to unnecessary multi-agent chemotherapy.

Excellent prognosis.

Tumor cells are derived from activated T cells that express CD30 antigen.

Disease specific survival rate at 5 years is 85-90 percent (Liu HJL).

Brentuximab is the first treatment specifically indicated for anaplastic large cell lymphoma.

Brentuximab in anaplastic large cell lymphoma, a trial of 58 patient’s was associated with a 86% response rate and a complete remission rate of 53% with a median duration of response of 12.6 months.

 

Crizotinib, a tyrosine kinase inhibitor with activity against ALK, ROS1, and MET was granted FDA approval for use in pediatric patients with relapsed or refractory ALK-positive anaplastic large cell lymphoma.

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