Management of Peripheral T-Cell Lymphoma in Children and Adolescents Including STAT 3 Mutation Hyper-IgE Syndrome: One Size Does Not Fit All.

Peripheral T-cell lymphoma (PTCL) is a rare form of lymphoma in children with limited published data on treatment and lack of a uniformly accepted treatment algorithm. We retrospectively analyzed the data in children up to 18 years of age diagnosed to have PTCL from January 2016 to June 2020. The study included six children with a median age of 10 years, the youngest being a 7-month-old girl. According to the WHO-PTCL classification, three had PTCL-not otherwise specified (NOS), 2 had hepatosplenic TCL, and 1 had subcutaneous panniculitis-like TCL. All children had presented with advanced disease, 4 in St. Jude stage IV, 2 in St. Jude stage III. Three children received CHOEP chemotherapy including cyclophosphamide, doxorubicin, vincristine, prednisone, etoposide, while 1 child received CHOP. Two children received induction as per acute lymphoblastic leukemia followed by Bendamustine. Two patients succumbed to progressive disease, the infant with PTCL-NOS and 1 child with hepatosplenic TCL. Three children were in remission (median follow up of 44 mo). One child with PTCL-NOS Stage IV had an underlying STAT3 mutated hyperimmunoglobulin E syndrome and was in remission 12 months post a matched unrelated donor hematopoietic stem cell transplantation. He had grade 4 skin graft versus host disease and required extracorporeal photopheresis and ibrutinib, to which he had responded. CHOEP chemotherapy is well-tolerated and subcutaneous panniculitis-like TCL has the best prognosis thus far.