Important note: Information in this article was accurate in 1992. The state of the art may have changed since the publication date.
BURKITT'S LYMPHOMA AND BURKITT CELL ACUTE LEUKEMIA IN ADULTS (MEETING ABSTRACT)
Haematologica; 76(Suppl 4):67 1991. Unique Identifier : AIDSLINE ICDB/92680567 Brusamolino E; Pagnucco G; Bernasconi P; Orlandi E; Malago D; Lazzarino M; Bernasconi C; Cattedra di Ematologia, Universita di Pavia Divisione di; Ematologia, Ospedale Policlinico San Matteo IRCCS, Pavia 27100,; Italy
Abstract:
We have retrospectively analyzed the characteristics of 24 adult patients (pts) affected with nonendemic Burkitt's lymphoma (BL) and Burkitt cell acute leukemia. 13 pts presented as lymphoma with nodal and/or extranodal masses and 11 showed a leukemic picture with or without tumoral mass. Burkitt cell leukemia was defined by L3 morphology of FAB classification (L3 ALL) and immunophenotype (B cell origin). Tests for HIV-antibodies were carried out in 16 pts after 1982, 5 of them (21%) showed HIV-positive. The median age of the entire group was 29 yr with a male preponderance (75%). Abdominal masses were present in 5/13 BL pts (38%); 8 presented with Stage IV and 4 had bone marrow disease (31%). Median LDH value was 580 mU/mL. CNS was involved in 25% of pts (6/24) at diagnosis. SIg were present on blast of all L3 ALL cases and CALLA was positive in half of them; no myeloid antigens were expressed. In all cases studies for karyotype, the t(8;14) translocation was evident. As of therapy, 5 pts were not evaluable due to early death, 12 (7 L3 ALL and 5 BL) were given an intensive ALL program including intrathecal CNS prophylaxis, 5 a cyclic conventional chemotherapy (CT) and 2 pts with bulky extranodal presentation (jaw and CNS) have entered a new protocol alternating high-dose cyclophosphamide, high-dose cytarabine with mitoxantrone, and the CHOP regimen. The overall complete remission rate was 42%: 5/12 pts given ALL therapy, 1/5 given conventional CT and both cases receiving the new protocol. Among 8 remitters, 5 have relapsed so far within a median time of 6 mo. Three of 13 (23%) pts with lymphoma presentation are long-remitters and may be cured; all had Stage II disease and low LDH at diagnosis (less than 250 mU/ml). No pts with L3 ALL are surviving. All HIV-positive pts did very poorly with a median survival of 4 mo. CNS disease (8 cases) and septic shock (6 cases) were the most frequent terminal events in the entire series. In our experience, the prognosis of advanced stage BL and L3 ALL in adults remains dismal; the high prevalence of CNS disease and HIV-positivity has contributed to the poor outcome. A new intensive protocol with alternating high-dose drugs (cyclophosphamide and cytarabine) and CNS prophylaxis is on-going.
Keywords: Adult Antigens, Neoplasm/ANALYSIS Antineoplastic Agents, Combined/THERAPEUTIC USE *Burkitt's Lymphoma Cyclophosphamide/ADMINISTRATION & DOSAGE/THERAPEUTIC USE Doxorubicin/THERAPEUTIC USE Female Human HIV Infections/COMPLICATIONS Karyotyping *Leukemia, B-Cell, Acute Male Mitoxantrone/ADMINISTRATION & DOSAGE Phenotype Prednisone/THERAPEUTIC USE Retrospective Studies Translocation (Genetics) Vincristine/THERAPEUTIC USE ABSTRACT 920630
M9261011
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Important note: Information in this article was accurate in 1992. The state of the art may have changed since the publication date.
