A CD30- positive primary cutaneous T-cell lymphoma.
An aggressive peripheral T cell lymphoma derived from cytotoxic T-cells that strongly and uniformly expressed CD30.
Accounts for fewer than 5% of all NHLs.
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.
Anaplastic lymphoma kinase positive and anaplastic lymphoma kinase negative tumors in anaplastic large cell lymphoma must be distinguished from one another because they have vastly different prognoses, with alkaline anaplastic lymphoma kinase positive cases being favorable.
There are four subtypes of ALCL: systemic ALCL, ALK positive systemic ALCL, ALK negative breast implants associated ALCL, and primary cutaneous ALCL.
ALCL, ALK positive is most common in children and young adults and is characterized by the over expression of ALK-1 protein, resulting from chromosomal translocation t 2:5 in 40 to 60% of patients.
Most patients with systemic ALCL present with advanced stage III or IV disease:frequent association with symptoms and extranodal involvement.
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.
Breast implants associated anaplastic large cell lymphoma is an extremely rare sub type of T cell non-Hodgkin’s lymphoma that mostly behaves indolently and is localized to the breast implant region: he can’t be cured with surgery alone in the vast majority of cases.
The median time from placement of the implant to the development of a breast induced angioplastic large cell lymphoma is 7 to 11 years: the cases have been seen as soon as 2.2 months and as long as 44 years from the initial implant placement.