Lymphoblastic lymphoma in adults.
Understanding of the pathogenesis and biology of precursor T-cell and B-cell neoplasms has advanced significantly with the description of gene expression profiling studies, especially in T-cell disease. These studies have demonstrated leukemic arrest at various stages of thymocyte maturation, characterized by gene expression signatures with prognostic significance. Optimal treatment strategies for adult lymphoblastic lymphoma are uncertain, although current evidence supports the use of regimens similar to those used in acute lymphoblastic leukemia, with intensive induction therapy, central nervous system prophylaxis, and prolonged consolidation maintenance therapy. Current studies do not demonstrate a benefit from stem cell transplantation in first remission, although this approach is probably beneficial in relapsed disease. Identification of new therapeutic targets by further molecular studies is required.
Sweetenham
John W
JW
Hematology & Oncology/R35, Cleveland Clinic Foundation, OH 44195, USA. sweetej@ccf.org
D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001402:B-Lymphocytes / Q000473:pathology; D002648:Child; D003131:Combined Modality Therapy; D015972:Gene Expression Regulation, Neoplastic; D015321:Gene Rearrangement; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D008479:Mediastinal Neoplasms / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy; D015337:Multicenter Studies as Topic / Q000706:statistics & numerical data; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy / Q000209:etiology / Q000235:genetics / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery; D011379:Prognosis; D016032:Randomized Controlled Trials as Topic / Q000706:statistics & numerical data; D012074:Remission Induction; D016879:Salvage Therapy; D013601:T-Lymphocytes / Q000473:pathology; D016896:Treatment Outcome; D055815:Young Adult