ABSTRACT
Fifty-three consecutive cases of adult CD30+ anaplastic large cell lymphoma (ALCL) have been analyzed. Thirty-six were classified as Hodgkin's disease like variety (HL) (67%) and seventeen as so-called common type (CT) (33%). All cases strongly expressed the CD30/Ki-1 antigen; the neoplastic cells expressed CD15, CD45 and EMA in 60%, 44% and 33% of cases, respectively; T. B and null phenotypes were found in 37%, 17% and 46% of cases. Bulky mediastinal, B symptoms, and extranodal disease at diagnosis were present in 36%, 49% and 25% of cases. EBV encoded latent membrane protein (LMP-1) was found in 10 cases. Of the 13 tested cases only 4 expressed a weak positivity of the CD40 molecule, in a fraction of the tumor cells; in the same cases CD21 was never found. Patients were treated with various protocols; of the 50 evaluable patients, 39 (78%) obtained a complete remission (CR), 3 (6%) a partial remission (PR) and 8 (16%) did not respond. The projected overall disease free survival (DFS) at 36 months is 70%. Only patients with advanced disease stage (III-IV) showed a statistically decreased DFS and survival. Only symptomatic and extranodal disease significantly appeared to influence survival. This study confirms the good outcome of this group of lymphomas and differs from other reports for some clinical (lower percentage of advanced stage, extranodal disease and skin infiltration) and pathological (HL/CT ratio and immunophenotype) features.
Subject(s)
Lymphoma, Large-Cell, Anaplastic/pathology , Adult , Aged , Aged, 80 and over , Diagnosis, Differential , Female , Hodgkin Disease/diagnosis , Humans , Immunophenotyping , Lymphoma, Large-Cell, Anaplastic/diagnosis , Lymphoma, Large-Cell, Anaplastic/therapy , Male , Middle Aged , Phenotype , Retrospective Studies , Treatment OutcomeSubject(s)
AIDS-Related Complex/complications , Acquired Immunodeficiency Syndrome/complications , Autoimmune Diseases/etiology , Hematologic Diseases/etiology , AIDS-Related Complex/blood , AIDS-Related Complex/immunology , Acquired Immunodeficiency Syndrome/blood , Acquired Immunodeficiency Syndrome/immunology , Anemia, Aplastic/etiology , Anemia, Aplastic/therapy , Autoimmune Diseases/immunology , Autoimmune Diseases/therapy , Blood Platelets/immunology , Female , Hematologic Diseases/immunology , Hematologic Diseases/therapy , Humans , Male , Thrombocytopenia/etiology , Thrombocytopenia/immunologyABSTRACT
This is a report from a cooperative study on hairy cell leukemia (HCL) involving 20 Hematology Departments in Italy. Data for the patients was collected between January 1967 and December 1981 and included 235 cases of which 203 could be evaluated; 160 were males (78.8%) and 43 females (21.2%) with an M:F ratio of about 3:1; mean age was 54 years (range 26-82 yrs). The diagnostic criteria of admission were: typical aspect of hairy cells, in peripheral blood and bone marrow smears, tartrate resistant acid phosphatase (TRAP) positivity, typical bone marrow, spleen, liver and/or lymph node histology, and/or electronmicroscopy. On the basis of hemoglobin level and spleen size at the time of diagnosis, three stages could be distinguished according to Jansen: 51 patients, 27 of which splenectomized, were in stage I; 67 patients, of which 44 splenectomized, were in stage II; 85 patients of which 60 splenectomized, were in stage III. The actuarial survival curves of these patients showed clear distinction between the three stages. In the first stage the difference in survival, between splenectomized and nonsplenectomized groups, was not statistically significant (p less than 0.5): on the contrary, in stages II and III the difference in survival was statistically significant (stages II and III; p less than 0.01).
