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Int J Hematol (2008) 88:299303 DOI 10.1007/s12185-008-0160-x
CASE REPORT
Aggressive CD5-positive B-cell lymphoma after remissionof CD5-negative follicular lymphoma with distinct immunoglobulin heavy chain rearrangement and translocation
Takashi Sonoki Sonoko Ishihara Shima Uneda Nobuyoshi Hanaoka Miwa Kurimoto Hiroshi Matsuoka Hideki Nakakuma
Received: 3 June 2008 / Revised: 4 August 2008 / Accepted: 4 August 2008 / Published online: 30 August 2008 The Japanese Society of Hematology 2008
Abstract We report a unique, aggressive B-cell lymphoma that developed after the long-term remission of follicular lymphoma (FL). FL cells were negative for CD5, whereas aggressive lymphoma cells were positive for CD5. In FL, one immunoglobulin heavy chain gene (IGH) allele underwent V/D/J recombination and another t(14;18) (q32;q21). In aggressive lymphoma, one IGH allele underwent D/J recombination and another translocation, but not t(14;18)(q32;q21). An aggressive lymphoma-specic D/J sequence was detected in FL tissue. Our results indicated that the two tumors arose from distinct B cells and that they existed concurrently in the same lymph node.
Keywords Secondary lymphoma Follicular
lymphoma CD5 t(14;18)(q32;q21) Immunoglobulin
heavy chain gene
1 Introduction
Follicular lymphoma (FL) is the second most common non-Hodgkin lymphoma, which generally shows an indolent clinical course. The tumor cells are positive for CD10, CD19, and CD20, and negative for CD5. The vast majority of follicular lymphomas show a specic translocation,
t(14;18)(q32;q21), resulting from a reciprocal translocation between the immunoglobulin heavy chain gene (IGH) on 14q32 and the anti-apoptotic gene, BCL2, on 18q21, which occurs at the early stage of B-cell differentiation in bone marrow (BM). Although this chromosome translocation confers anti-apoptotic properties on affected B cells, this event is insufcient for full oncogenesis. Recently, the lymph node microenvironment has been shown to participate in the oncogenesis of FL [1].
Secondary transformation to a more aggressive lymphoma from primary FL can occur during the clinical course. In addition to clinical or morphologic transformation, phenotypic changes, such as gain of the CD5 antigen, rarely occur [2]. Despite morphologic and phenotypic alterations, the vast majority of transformed tumor cells share the same IGH rearrangement or retain the t(14:18)(q32;q21) translocation with primary FL cells. This shows that the transformed tumor originates from a common B-cell clone of the primary FL [3].
Herein, we report an aggressive B-cell lymphoma which appeared four years after the...