T-cell/histiocyte-rich large B cell lymphoma

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 
         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 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 (lt10) 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
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 gt80 for stage I II)
10
(No Transcript)
11
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
  lymphocytepredominant Hodgkin lymphoma with
• nodules resembling T-cell/histiocyte-rich
  B-cell lymphoma differential diagnosis between
• nodular lymphocytepredominant Hodgkin
  lymphoma and T-cell/histiocyte-rich B-cell
• lymphoma. Blood. 20031023753-3758.
• 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) 14411421