T-cell/histiocyte-rich large B cell lymphoma Monirath Hav, MD, PhD fellow Pathology Department Ghent...
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Transcript of T-cell/histiocyte-rich large B cell lymphoma Monirath Hav, MD, PhD fellow Pathology Department Ghent...
T-cell/histiocyte-rich large B cell lymphoma
Monirath Hav, MD, PhD fellowPathology Department
Ghent University Hospital
Diffuse large B-cell lymphoma (DLBCL), NOS
T-cell/histiocyte rich large B-cell lymphoma
Primary DLBCL of the CNS
Primary cutaneous DLBCL, leg type
EBV+ DLBCL of the elderly*
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues2008 edition
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
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
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
Differential diagnosis
Main DD: NLPHL rosettes of T cells around tumour cells, usually no BM involvement, CD57+, small B cells in the background
Prognosis
• Aggressive lymphoma
• Refractory to presently available therapy
• IPI score is the only parameter of prognostic significance
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 (–)
LHRLBCL Vs NLPHL - differences
LHRLBCL NLPHL- CD20 + small reactive B cells are rare or absent in the background- CD8 + cells & histiocytes are common
- CD3 + cells do not form rosettes around tumour cells
- CD21+ FDC are absent- BM, spleen or liver involvement is frequent
- Aggressive lymphoma, refractory to current therapy
- CD20 + small reactive B cells are numerous in the background
- CD4 and CD57 + cells are common
- CD3 & CD57 + cells form rosettes around tumour cells
- CD21+ FDC form expanded meshwork
- BM, spleen, or liver involvement is rare
- Non agressive lymphoma, responsive to therapy (10 y OS >80% for stage I & II)
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