Abstract

Splenic marginal zone lymphoma (SMZL) is a low-grade lymphoma showing a rather nonspecific immunophenotype. Gene expression profiling studies suggested that TCL1A could be a marker of SMZL, but reported data are conflicting. We evaluated TCL1A expression in a series of spleen and bone marrow samples involved by SMZL and correlated the findings with other immunophenotypical, morphological, and clinical data. In addition, we evaluated the expression of TCL1A in a series of spleens and lymph nodes involved by lymphomas that might mimic SMZL (13 nodal marginal zone lymphomas (NMZL), 39 follicular lymphomas (FL), 30 B-cell chronic lymphocytic leukemias (B-CLL), 31 mantle cell lymphomas (MCL), 1 lymphoplasmacytic lymphoma) and 15 bone marrow specimens involving hairy cell leukemia (HCL). TCL1A staining was negative in 24/31 cases of SMZL (77 %); 27/31 MCL and all B-CLL were positive for TCL1A; 32/34 cases of nodal FL (96 %) and all five splenic FL were positive for TCL1A, although at a lower intensity. Eight of 13 NMZL were positive for TCL1A, often showing a heterogeneous staining pattern. All HCL samples were strongly positive for TCL1A. No correlation was found between the pattern of splenic infiltration, TCL1A expression, and the clinical course. TCL1A-positive SMZL showed a higher rate of DBA44 staining compared to the negative ones, and this difference was statistically significant (Fisher test, single-tailed, p = 0.0397). Our data support the use of TCL1A in the panel of diagnostic markers used in the differential diagnosis of splenic low-grade B-cell lymphoma; a possible prognostic value, however, needs a larger series to be established.

Highlights

  • Splenic B-cell marginal zone lymphoma is a rare entity, accounting for less than 2 % of all lymphoid malignancies; it shows an indolent clinical course, 10 % of cases progress to diffuse large B cell lymphoma [1]

  • A commercially available specific marker of SMZL is lacking, and the immunophenotypical profile of SMZL is currently mainly defined on the basis on the lack of expression of markers associated with its mimickers, including CD5, CD10, CD23, cyclin D1, CD25, CD11c, and Annexin A1 to name just a few

  • Nine cases of non-Hodgkin lymphoma involving the spleen were used as controls, including five follicular lymphomas (FL), one mantle cell lymphoma (MCL), one small Bcell lymphoma/chronic lymphocytic leukemia (B-CLL/SLL), one hairy cell leukemia (HCL), and one lymphoplasmacytic lymphoma (LPL)

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Summary

Introduction

Splenic B-cell marginal zone lymphoma is a rare entity, accounting for less than 2 % of all lymphoid malignancies; it shows an indolent clinical course, 10 % of cases progress to diffuse large B cell lymphoma [1]. A commercially available specific marker of SMZL is lacking, and the immunophenotypical profile of SMZL is currently mainly defined on the basis on the lack of expression of markers associated with its mimickers (follicular lymphoma, mantle cell lymphoma, B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma, hairy cell leukemia), including CD5, CD10, CD23, cyclin D1, CD25, CD11c, and Annexin A1 to name just a few. Myeloid cell nuclear differentiation antigen (MNDA) has recently been described as a sensitive ( not completely specific) marker of nodal and splenic marginal zone lymphomas, that might be useful both in primary sites and in the bone marrow [8]

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