Abstract
Therapy of lymphoblastic lymphoma (LL) has evolved with use of chemotherapy regimens modeled after those for acute lymphocytic leukemia (ALL). We treated 33 patients with LL with the intensive chemotherapy regimens hyper-CVAD (fractionated cyclophosphamide, vincristine, Adriamycin, and dexamethasone) or modified hyper-CVAD used for ALL at our institution. Induction consolidation was administered with 8 or 9 alternating cycles of chemotherapy over 5 to 6 months with intrathecal chemotherapy prophylaxis, followed by maintenance therapy. Consolidative radiation therapy was given to patients with mediastinal disease at presentation. No consolidation with autologous or allogeneic stem cell transplantation was performed. At diagnosis, 80% were T-cell immunophenotype, 70% were stages III to IV, 70% had mediastinal involvement, and 9% had central nervous system (CNS) disease. Of the patients, 30 (91%) achieved complete remission, and 3 (9%) achieved partial response. Within a median of 13 months, 10 patients (30%) relapsed or progressed. Estimates for 3-year progression-free and overall survival for the 33 patients were 66% and 70%, respectively. Estimates for the patients with known T-cell immunophenotype were 62% and 67%, respectively. No parameters (eg, age, stage, serum lactate dehydrogenase [LDH], {beta}2 microglobulin) appeared to influence outcome except for CNS disease at presentation. Modification of the hyper-CVAD regimen with anthracycline intensification did not improve outcome. Other modifications of the program could include incorporation of monoclonal antibodies and/or nucleoside analogs, particularly for slow responders or those with residual mediastinal disease. (Blood. 2004;104:1624-1630)
Introduction
Lymphoblastic lymphoma (LL) represents 2% to 4% of adult non-Hodgkin lymphomas (NHLs).1 This high-grade lymphoma has several distinct clinicopathologic features. Clinically, it often presents with mediastinal and supradiaphragmatic
lymph node involvement, manifesting as cough, shortness of breath, respiratory distress, and/or superior vena cava (SVC) syndrome.2-6 Central nervous system (CNS) involvement at presentation occurs in 20% of cases and is a frequent site of relapse in the absence of CNS prophylaxis.2-6 Morphologically, the lymphoblasts are medium-to-large in size and have a typical convoluted nuclei appearance, staining brightly for terminal deoxynucletidyl transferase (TdT).4-7 Higher mitotic rates are observed when compared with French-American-British (FAB) L1 or L2 lymphoblasts.
When evaluated by immunophenotyping, most LLs are of T-cell origin. Non-T-cell LL occurs in 5% to 20% of cases, often has a different clinical presentation (younger age, female preponderance, higher incidence of skin involvement), and has a better prognosis.8 When LL involves the marrow or progresses to a leukemic phase (> 25% marrow blasts), it may be indistinguishable from T-cell acute lymphocytic leukemia (ALL). Although these disorders likely represent the same spectrum of disease with different presentations, features such as presence or absence of lymph-adenopathy, splenomegaly, and/or thrombocytopenia may differentiate the 2 entities and further delineate prognosis.1,9,10
Prior to the application of intensive ALL regimens to LL, prognosis was poor. With lymphoma-like regimens such as CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) and asparaginase, complete response (CR) rates ranged from 40% to 70% with disease-free survival (DFS) rates of 20% to 50%.
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