Anaplastic Large Cell Lymphoma: A Clinicopathologic Study of 53 Patients

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.

ALK-Negative Anaplastic Large Cell Lymphoma and Hodgkin’s Disease of Nodular Sclerosing Type in 2 Siblings

“…Like the cases described in previous trials [36, 37], the present case of ALK negative, anaplastic large cell lymphoma in line with its borderline status, responded to ABVD chemotherapy (scheme specific for HD) and local radiotherapy.…”

High-dose therapy with autologous stem cell transplantation in patients with peripheral T cell lymphomas

“…[29][30][31] Earlier reports on the use of HDT have been sparse and concern few cases treated in relapse. 30,[32][33][34] One study has tested HDT as 'upfront' consolidation in chemosensitive ALCL in order to sustain first CR and prevent relapse. 25 With this approach they achieved a CR rate above 90% and no late relapses.…”

Autologous stem cell transplantation for T and null cell CD30-positive anaplastic large cell lymphoma: analysis of 64 adult and paediatric cases reported to the European Group for Blood and Marrow Transplantation (EBMT)

“…11,12 It is surprising that although the clinical, histological and immunophenotypic characteristics indicate that this lymphoma may be a group of aggressive lymphoid tumours, reports on autologous stem cell transplantation (ASCT) in ALCL are occasional, and mainly refer to cases in relapse. 1,2,4,5 It was recently shown, in a study performed on a small monocentric series, that primary systemic CD30-positive ALCL of the adults is a curable disease when ASCT is performed as part of first-line treatment. 13,14 In this study patients with B cell ALCL were included.…”

“…[1][2][3][4][5][6][7][8] Relapse rate ranges widely from 25 to 45%, with less than 50% of patients alive and free of disease at a median observation time from diagnosis no longer than 36 months. [1][2][3][4][5][6][7][8] The different behaviour in relapse rate may reflect the existence of a heterogeneous family of diseases: ALCL T and null cell common type, ALCL T and null cell Hodgkin-like and diffuse anaplastic large B cell lymphoma. 9,10 These three major entities probably have different prognosis and outcome, but this is not clearly underlined in the papers already published on this subject.…”