1988 Volume 2 Issue 4 Pages 411-417
The designation lymphoma syndrome leukemia (LSL) is applied to childhood atypical acute lymphoblastic leukemia (ALL) associated with lymphoma features. There is much controversy about whether LSL and lymphoblastic lymphoma, another representative disease of T-lymphoid malignancy, constitute and identical clinicopathological entity or not. We described here clinical features of 3 cases with LSL. All of them initially presented with massive mediastinal mass and other lymphoma-like lesions but no anemia or thrombocytopenia. Despite the induction of complete remission by conventional chemotherapy, early relapses consistently occurred. These cases became resistant to further chemotherapies, and eventually died of diffuse extramedullary involvements within 36 months after the onset. Leukemic blasts from the 3 cases were classified into French-American-British (FAB) L2 subtype of ALL, but the appearance of nuclei, nuclear chromatin, and cytoplasm somehow differed from those of leukemic blasts from typical ALL cases. Immunophenotyping showed that the leukemic blasts from the 3 cases were of T-cell lineage. LSL seems to represent a distinct clinical entity of ALL in childhood with absolutely poor prognosis, suggesting that very intensive chemotherapy or bone marrow transplantation has to be introduced as the initial treatment for this condition.
