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

Sixty-four adult patients with lymphoblasts 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 less than 0.0005), symptoms (P less than 0.025), bulky disease (P less than 0.025) and bone marrow involvement (P less than 0.025). Furthermore the response to therapy was strongly correlated with prognosis (P less than 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.