Lymphoblastic lymphoma in adolescents and adults. Clinical, pathological and prognostic evaluation

Sixty-four adult patients with lymphoblastic lymphoma (LB) identified according to Kiel Classification were analyzed retrospectively. Three distinct clinical presentations were identified: prevalent abdominal disease ( 29 pts = 45.3%), prevalent mediastinal disease ( 14 pts = 21.9%) and prevalent superficial node involvement ( 21 pts = 32.8%). On histological grounds, the patients with abdominal disease were mainly associated to “Burkitt like” cell lymphoma ( 55%); patients with mediastinal disease to convoluted cell type ( 58%); and those with superficial node disease to unclassified cell type ( 48%). Immunological studies showed a significant correlation between mediastinal disease and T phenotype ( P = 0.0011), abdominal disease and B phenotype ( P = 0.00042), and between superficial node disease and non-B non-T phenotype ( P = 0.00024). Survival was independent of the type of clinical presentation and protocol employed but was correlated with the stage ( P < 0.0005), symptoms ( P < 0.025), bulky disease ( P < 0.025) and bone marrow involvement ( P < 0.025). Furthermore the response to therapy was strongly correlated with prognosis ( P < 0.0001) with 34.5 months median survival for complete responders, 9 months for partial responders, and 3 months for non-responders. Four patients underwent bone marrow transplantation (three autologous and one allogeneic BMT in a patient in leukemic phase); three of them are still in CR ( 18, 22 and 27 months from the transplant) while one patient had an early relapse and died 3 months later.