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T-cell/histiocyte-rich large B cell lymphoma

T-cell/histiocyte-rich large B cell lymphoma. Monirath Hav, MD, PhD fellow Pathology Department Ghent University Hospital.    Diffuse large B-cell lymphoma (DLBCL), NOS. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues 2008 edition. Introduction.

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T-cell/histiocyte-rich large B cell lymphoma

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  1. T-cell/histiocyte-rich large B cell lymphoma Monirath Hav, MD, PhD fellow Pathology Department Ghent University Hospital

  2.    Diffuse large B-cell lymphoma (DLBCL), NOS WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues 2008 edition

  3. Introduction • Characterized by a limited number of scatterd, large, atypical B cells embedded in a background of abundant small T cells and frequently Histiocytes • Age: mainly middle-aged men (12-61 yo, 75% cases are male) • Refractory to present chemotherapy • Postulated normal counterpart: germinal center B cell

  4. Morphology • Diffuse or less commonly vaguley nodular growth pattern • Scattered, single, large B cells (<10%) embedded in the background of small T lymphocytes and variable numbers of histiocytes • Tumour cells do not form aggregrates or sheets and mimic popcorn cells in NLPHL but usually show a more pronouced variation in size • Cases in which tumour cells form sheets or nests should be classified as a subtype of DLBCL, NOS • Eosinophils and plasma cells are not found

  5. Immunophenotype • The large atypical cells are: • Positive for pan B cell markers, BCL6 & some for BCL2 & EMA • Negative for CD15, CD30, & CD138 • The background is composed of CD68+ histiocytes and CD3, CD5 & CD8 + T cells • Unlike in NLPHL, rosettes of T-cells around the tumour cells are not found in THRLBCL

  6. Differential diagnosis  Main DD: NLPHL  rosettes of T cells around tumour cells, usually no BM involvement, CD57+, small B cells in the background

  7. Prognosis • Aggressive lymphoma • Refractory to presently available therapy • IPI score is the only parameter of prognostic significance

  8. LHRLBCL Vs NLPHL - similarities • Predilection for middle-aged men • Both derived from germinal center B cells • Diffuse or vague nodularity growth pattern • Eosinophils and plasma cells are seldom seen • Large atypical tumour cells are (+) for CD20, CD79a, PAX5, CD45, CBL6, & BCL2 in the background of small CD3 + cells • Large atypical tumour cells are (–) for CD30* & CD15 • Large atypical tumour cells are usually EBV (–)

  9. LHRLBCL Vs NLPHL - differences

  10. References 1. WHO classification of Tumours of Haematopoietic and Lymphoid Tissues, 2008 edition 2. Frank X. Zhao. Nodular Lymphocyte-Predominant Hodgkin Lymphoma or T cell/Histiocyte Rich Large B-cell Lymphoma: The Problem in “Grey Zone” Lymphomas. Int J Clin Exp Pathol (2008) 1, 300-305 3. Ludmila Boudova et al. Nodular lymphocyte–predominant Hodgkin lymphoma with nodules resembling T-cell/histiocyte-rich B-cell lymphoma: differential diagnosis between nodular lymphocyte–predominant Hodgkin lymphoma and T-cell/histiocyte-rich B-cell lymphoma. Blood. 2003;102:3753-3758. 4. Megan S. Lim et al. T-Cell/Histiocyte-Rich Large B-Cell Lymphoma: A Heterogeneous Entity With Derivation From Germinal Center B Cells. Am J Surg Pathol 26(11): 1458- 1466, 2002. 5. Á. Illés et al. Nodular Lymphocyte Predominant Hodgkin Lymphoma (NLPHL) Clinicopathological Features Based on the Data of Two Hungarian Lymphoma Centres. Pathol. Oncol. Res. (2008) 14:411–421

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