Anaplastic large cell lymphoma (Ki‐1+/CD30+) in childhood

Abstract

In 1985, Stein et al. (Blood 66:848-858) described a large cell lymphoma consisting of activated lymphoid elements, expressing the Reed-Sternberg cell-associated antigen Ki-1 (CD30); this tumor has recently been included in the updated Kiel classification and has been termed anaplastic large cell lymphoma (ALCL). This report concerns 13 patients with previously untreated ALCL who were admitted to the Pediatric Hematology and Oncology Department of Bologna University between January 1983 and December 1989. Ten cases were diagnosed as common type ALCL, 1 as Hodgkin's-related ALCL and the remaining 2 as histiocyte-rich ALCL/lymphohistiocytic T-cell lymphoma. T-cell markers were present in 85% of the cases, and B phenotype in the remaining 15%. About one-half of the patients presented systemic symptoms; all but one showed more than one involved site, mostly an association of nodal and extranodal sites. All patients (1 in stage I, 7 in stage II, and 5 in stage III) were treated with a modified version of the LSA2-L2 protocol. Survival and event-free survival at 4 years were 100% and 62.9%, respectively. Two out of 3 relapsed patients presented a recurrence of disease when they were off-therapy 24 and 36 months after first complete remission. Good response to salvage chemotherapy of all the relapsed patients and the long duration of the second complete remission were a distinctive characteristic of this neoplasm when compared to other histological subtypes. The clinical presentation, the tendency to develop late relapses and to achieve and maintain second remission easily suggest that ALCL is a high grade non-Hodgkin's lymphoma, from the histological point of view, but is similar to Hodgkin's disease from the clinical point of view. This would confirm the hypothesis that Hodgkin's disease and ALCL represent a continuous spectrum of the same disease.

Pathobiology of NPM-ALK and variant fusion genes in anaplastic large cell lymphoma and other lymphomas

“…The common/classical type is by far the most frequent form, Incidence rates were calculated from data published in 10 studies on a total of 598 ALCL patients. 17,27,30,34,47,49,50,63,76,77 Indicated are the means of all cases combined and in parentheses the range of incidence rates between the various studies (not all studies included the same variants). b Detailed descriptions of the ALCL variants are found in the references indicated.…”

Prognostic factors in childhood anaplastic large cell lymphoma: results of a large European intergroup study

“…10,11 Finally, in other groups and especially in adults, ALCL patients receive repeated-pulse chemotherapy according to protocols designed for all large-cell lymphomas, regardless of the immunophenotype, including large B-cell lymphoma and ALCL. [12][13][14] In published pediatric series, [6][7][8][9]11,[15][16][17][18][19][20] the analysis of prognosis factors has been limited to date because of the small numbers of patients.…”

Successful treatment of secondary acute myeloid leukemia relapsing after allogeneic bone marrow transplantation with donor lymphocyte infusion failed to prevent recurrence of primary disease: a case report

“…After an LSA2-L2-like induction and consolidation courses (Table 1), the patient was treated with maintenance phase I (weekly, alternating a couple of drugs) until completion of the 12 months of therapy, and maintenance phase II (weekly methotrexate and daily 6-mercaptopurin) for a total 24 months of therapy. 6 He obtained prompt relief from pain, followed by a reduction in the osteolytic lesions. At the end of consolidation treatment, he was considered to be in clinical remission with a clear improvement in the bone lesions on skeletal 99 Tc scan.…”