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Prepublished online as a Blood First Edition Paper on February 27, 2003; DOI 10.1182/blood-2002-06-1864. This Article Full Text Full Text (PDF) All Versions of this Article: 2002-06-1864v1 101/12/4975    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Orchard, J. Articles by Oscier, D. Search for Related Content PubMed PubMed Citation Articles by Orchard, J. Articles by Oscier, D. Related Collections Neoplasia Oncogenes and Tumor Suppressors Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  Blood, 15 June 2003, Vol. 101, No. 12, pp. 4975-4981 NEOPLASIA A subset of t(11;14) lymphoma with mantle cell features displays mutated IgVH genes and includes patients with good prognosis, nonnodal disease Jenny Orchard, Richard Garand, Zadie Davis, Gavin Babbage, Surinder Sahota, Estella Matutes, Daniel Catovsky, Peter W. Thomas, Hervé Avet-Loiseau, and David Oscier From the Department of Haematology, Royal Bournemouth Hospital, United Kingdom; Laboratory of Haematology, University Hospitals, Nantes, France; Tenovus Research Laboratory, University Hospitals, Southampton, United Kingdom; Academic Department of Haematology and Cytogenetics, Royal Marsden NHS Trust, London, United Kingdom; and Dorset Research and Development Support Unit, Poole Hospital, United Kingdom. We analyzed lymphocyte morphology, histology, immunophenotype, immunoglobulin heavy chain (IgVH) gene mutations, and clinical course in 80 unselected patients presenting with circulating t(11;14) lymphocytes. Of the 80 patients, 43 had peripheral lymphadenopathy (nodal group), and histology confirmed mantle cell lymphoma (MCL) in all. There were 37 patients with no lymphadenopathy (nonnodal group); 13 of 37 had histology, all showing MCL. IgVH genes were unmutated in 28 (90%) of 31 nodal and 15 (44%) of 34 nonnodal cases (P = .0001); CD38 was positive in 32 (94%) of 34 nodal and 16 (48%) of 33 nonnodal cases (P < .001); 41 (95%) of 43 nodal patients required immediate treatment compared with 18 (49%) of 37 nonnodal patients who had indolent disease (P < .0001). Median survival (95% confidence interval) was 30 months (10-50) in the nodal group and 79 months (22-136) in the nonnodal group (P = .005). Mutation status did not statistically affect survival, but of 6 long-term survivors (> 90 months) all were nonnodal and 5 of 5 had mutated IgVH genes. Lymphocyte morphology was heterogeneous in both groups: typical MCL in 56 cases (34 nodal, 22 nonnodal), blastoid MCL in 8 cases (3 nodal, 5 nonnodal), and small-cell MCL in 16 cases (6 nodal, 10 nonnodal, P = .12). Matutes immunophenotyping score was 1 in 65 cases and 2 in 15 (8 nodal, 7 nonnodal). We find no evidence against a diagnosis of MCL in the nonnodal group and suggest that mutated IgVH genes may help identify patients with indolent disease. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: F. Forconi, E. Sozzi, E. Cencini, F. Zaja, T. Intermesoli, C. Stelitano, L. Rigacci, F. Gherlinzoni, R. Cantaffa, A. Baraldi, et al. 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