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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 (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 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. Social Bookmarking                   What's this? Submitted June 24, 2002 Accepted February 8, 2003 A sub-set of t(11;14) lymphoma with mantle cell features displays mutated IgVH genes and includes patients with good prognosis, non-nodal disease Jenny Orchard, Richard Garand, Zadie Davis, Gavin Babbage, Surinder Sahota, Estella Matutes, Daniel Catovsky, Peter W Thomas, Herve Avet-Loiseau, and David Oscier* Department of Hematology, Royal Bournemouth Hospital, Bournemouth, United Kingdom Laboratory of Hematology, University Hospitals, Nantes, France Tenovus Research Laboratory, University Hospitals, Southampton, United Kingdom Academic Department of Hematology and Cytogenetics, Royal Marsden NHS Trust, London, United Kingdom Dorset Research and Development Support Unit, Poole Hospital, Poole, United Kingdom * Corresponding author; email: david.oscier{at}lineone.net. Patients presenting with a t(11;14) lymphocytosis without peripheral lymphadenopathy frequently present diagnostic problems. We analyzed lymphocyte morphology, histology, immunophenotype, immunoglobulin heavy chain gene (IgVH) mutations, and clinical course in 80 unselected patients presenting with circulating t(11;14) lymphocytes. 43 patients had peripheral lymphadenopathy (nodal group) and in all histology confirmed MCL. 37 patients had no lymphadenopathy (non- nodal group); 13/37 had histology, all showing MCL. IgVH genes were unmutated in 28/31(90%) nodal and 15/34(44%) non-nodal cases (p=0.0001); CD38 was positive in 32/34(94%) nodal and 16/33(48%) non-nodal cases (p<0.001); 41/43 nodal patients required immediate treatment compared with 18/37(49%) non-nodal patients who had indolent disease(p<0.0001). Median survival (95% CI) was 30 months (10,50) in the nodal group and 79 months (22, 136) in the non-nodal group (p=0.005). Mutation status did not statistically affect survival but of 6 long-term survivors (>90 months) all were non-nodal and 5/5 had mutated IgVH genes. Lymphocyte morphology was heterogeneous in both groups: typical MCL in 56 cases (34 nodal, 22 non-nodal), blastoid MCL in 8 cases (3 nodal, 5 non-nodal) and small cell MCL in 16 cases (6 nodal, 10 non-nodal, p=0.12).No cases had morphology of SLVL, PLL, or atypical CLL. Matutes immunophenotyping score was 1 in 65 cases and 2 in 15(8 nodal, 7 non-nodal). IgVH gene usage showed no bias, in contrast to CLL or SLVL. We find no evidence against a diagnosis of MCL in the non-nodal 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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