KI-1 (CD30) ANAPLASTIC LARGE-CELL LYMPHOMA IN CHILDREN

Background: Ki-l (CD30) ALCL is a rare and distinct type of high-grade NHL. A relevant feature is young age at presentation. Most reported p ediatric series are retrospective analyses of heterogeneously-treated cases. Patients and methods: Between 1976 and 1993, Ki-l ALCL was diag nosed in 32 children (20 males, 12 females; median age 9 years; 10.4% of childhood NHL). Before 1987, original diagnoses had been malignant histiocytosis in 14 cases, and immunoblastic NHL in 2. The treatment p rogram for childhood T-lymphoblastic NHL, except for CNS prophylaxis, was applied in 28/32 cases. Radiotherapy to involved sites not in comp lete remission within 4 weeks after treatment start was used only prio r to 1987. Results: In 78% of cases, Ki-l ALCL presented in skin, bone , spleen and lung. Lymph nodes were frequently involved, often in asso ciation with periadenitis. Bone marrow and spinal fluid were negative in all cases. Eleven patients were classified as stage I/II, 21 as sta ge III. Fever was present in 65% of patients. Cell phenotype was T in 21/32, and null in the remaining 11. Treatment results were evaluable in 27/32 children, all of whom achieved complete remission. Seven rela psed and 4 died of their disease. Five-year survival and progression-f ree survival were 84% and 72%, respectively. Involvement of peripheral lymph nodes, absence of spleen, liver, lung and mediastinum involveme nt, and male sex were favourable indicators. Conclusions: Ki-l ALCL is a high-grade NHL with a relatively good prognosis when treated with m ultiagent intensive chemotherapy. Because of its clinical peculiaritie s a unique treatment approach is probably warranted.