CLINICOPATHOLOGICAL FEATURES AND TREATMENT OUTCOME OF CHILDREN WITH LARGE-CELL LYMPHOMA AND THE T(2,5)(P23,Q35)

The t(2;5)(p23;q35) was detected in 9 of the 18 cases of large-cell ly mphoma with an abnormal karyotype among 115 children with large-cell l ymphoma treated at St Jude Children's Research Hospital from 1975 to 1 993. When the cases containing the t(2;5) were classified according to the National Cancer Institute Working Formulation, 7 were large-cell, immunoblastic and 2 were diffuse large cell; according to the Kiel cl assification system, 6 were anaplastic large cell, 2 immunoblastic, an d 1 centroblastic. CD30 expression was documented in 6 of 8 cases test ed. All patients had nodal disease and 6 had extranodal involvement (b one in 4 cases and skin in 3). Eight of nine had advanced disease at d iagnosis (stage III in 7 and stage IV in 1). Complete remission (CR) w as attained in all patients and 6 remain in first CR for 19+ to 97+ mo nths. Three relapsed, but successfully obtained second remissions; 2 a re 58+ and 80+ months after retrieval therapy for local recurrences, a nd 1 patient died of recurrent disease. The t(2;5)(p23;q35) is associa ted with, but not limited to, anaplastic histology, a CD30(+) T-cell p henotype, advanced stage disease with nodal (+/-extranodal) involvemen t, and chemosensitivity at diagnosis and relapse. (C) 1994 by The Amer ican Society of Hematology.