Background: Ki-1 anaplastic large-cell lymphoma(ALCL) is characterized by mostly
large pleomorphic cells with a strong reactivity to the Ki-1(CD30) monoclonal antibody,
originally raised against the Reed-Stemberg cell line, and is included in the updated Kiel
classification for non-Hodgkin lymphoma (NHL) as a high-grade malignant NHL. We
studied the clinical features, immunohistochemistry, and clinical outcome of 5 cases of
Ki-1 ALCL in childhood.
Methods : We reviewed 5 cases of Ki-1 ALCL treated at the Department of Pediatrics,
Seoul National University Children's Hospital from Jan. 1992 to Dec. 1994.
Results : Three boys and two girls had a median age of 12 years at diagnosis. B
symptom was observed in one patient. Peripheral adenopathy was present in all patients,
while mediastinal adenopathy and retroperitoneal adenopathy occurred in two patients,
respectively. There were extranodal involvements in two patients; liver and bone.
Immunophenotypes were T cell in one patient and B cell in one patient; in the
remaining three patients, immunophenotype could not be determined. All patients were
treated with COMP regimen including cyclophosphamide, vincristine, methotrexate, and
prednisolone. Four patients have been in continuous complete remission for 14 months to
34 months, and all patients are alive.
Conclusion: Childhood Ki-1 ALCL is associated with the frequent extranodal
involvement, the lack of bone marrow disease, and the better overall survival.

Keywords: Ki-1 lymphoma, CD30, Anaplastic large cell lymphoma, Chemotherapy, Childhood