Lymphoma & related disorders
Mature B cell neoplasms
Large B cell lymphomas-special subtypes
T cell / histiocyte rich
Authors: Konrad Chan, M.D., Rumina Musani, M.D.
Last author update: 1 July 2017
Last staff update: 30 May 2023 (update in progress)
Copyright: 2003-2023, PathologyOutlines.com, Inc.
PubMed Search: T cell / histiocyte rich large B cell lymphoma
Table of Contents
Definition / general | Essential features | Diagrams / tables | Clinical features | Case reports | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Molecular / cytogenetics description | Differential diagnosis | Additional references | Board review style question #1 | Board review style answer #1Cite this page: Chan K, Musani R. T cell / histiocyte rich. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lymphomadiffusetcell.html. Accessed June 7th, 2023.
Definition / general
- Diffuse large B cell lymphoma subtype with abundant nonneoplastic T cells and histiocytes, that may obscure tumor cells
Essential features
- Must differentiate from nodular lymphocyte predominant Hodgkin lymphoma (NLPHL): has different microenvironment (cytotoxic T cells vs B cells / follicular dendritic (FD) meshwork); also requires clinical correlation (THRLBCL is advanced stage vs. NLPHL is indolent)
- Sample lymph node thoroughly to find typical areas of NLPHL in order to rule out THRLBCL
- Caution when diagnosing THRLBCL in a small biopsy: if there is history of NLPHL, describe as "THRLBCL-like transformation" of NLPHL
- Evidence exists that NLPHL and THRLBCL represent different spectrum of the same pathobiological entity
Diagrams / tables
Contributed by Konrad Chan, M.D.
Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) versus T cell / histiocyte rich B cell lymphoma (THRLBCL)
Clinical features
- Rare morphological variant of diffuse large B cell lymphoma (DLBCL); 1 - 3% of all B cell lymphomas
- Usually middle age adults, often with advanced stage (Ann Arbor III / IV) and involvement of lymph nodes, spleen, bone marrow
- Clinical presentation: fever, malaise, splenomegaly or hepatomegaly
- Same treatment protocol as DLBCL, NOS but lower complete remission rate
Case reports
- 32 year old man with a small bowel perforation (J Hematop 2010;3:29)
- 37 year old man with proptosis of the right eye (Ophthal Plast Reconstr Surg 2011;27:e31)
- 37 year old man with fever and pancytopenia (Case #317)
- 52 year old woman with THRLBCL of the thymus (Case Rep Hematol 2016;2016:2942594)
- 73 year old man with primary CNS tumor (Rare Tumors 2015;7:6084)
- CD5+ tumor (Mod Pathol 2002;15:1051, free full text)
- THRLBCL presenting on the skin (Dermatology 2011;222:225)
Gross images
Microscopic (histologic) description
- Diffuse or vaguely nodular pattern
- Scattered single neoplastic large B cells ( < 10% of all cells) amongst small reactive T cells and histiocytes
- Near absence of B lymphocytes in the background; no remnants of B follicles or clusters of small B lymphocytes
- Diverse morphologic and immunophenotypic features (Am J Surg Pathol 2002;26:1458)
- LP-like (lymphocyte predominant-like): pale and indistinct cytoplasm, lobated and vesicular nuclei resembling popcorn cells with small central nucleoli; background of small lymphocytes and often histiocytes, no granulomas
- Centroblast-like: pale eosinophilic cytoplasm, oval / round nuclei with vesicular chromatin and without nuclear atypia, small basophilic nucleoli adjacent to nuclear membrane
- HRS-like (Hodgkin Reed-Sternberg cell-like): large multinucleated cells with abundant amphophilic cytoplasm, pleomorphic nuclei with prominent eosinophilic or amphophilic round / central nucleoli
- Neoplastic B cells are surrounded by clusters of bland nonepithelioid histiocytes
Microscopic (histologic) images
Contributed by Drs. Asmaa Gaber Abdou and Nancy Youssef Asaad
Case #317
Various images
CD3
CD20
PAX5
CD30
Images hosted on other servers:
Low power view: retroperitoneal lymph node
Large cell population
Scattered eosinophils
THRLBCL
T cell component of NHL
CD20
Positive stains
Negative stains
Molecular / cytogenetics description
- PAX5 / IgH gene rearrangement by FISH
Differential diagnosis
- Classic Hodgkin lymphoma (cHL): neoplastic B cells are CD15+ / CD30+ (strong) / MUM1+ (strong); different microenvironment (see below)
- Immunodeficiency related lymphoproliferative disorder (ID-LPD): clinical history (primary immune disorder, posttransplant, immunosuppressive therapy); polymorphous (including plasma cells), EBER+
- Nodular lymphocyte predominant - Hodgkin lymphoma (NLPHL):
- Neoplastic B cells:
- BCL6- / MUM1+ favors THRLBCL
- IgD+ favors NLPHL
- Different microenvironments (see diagrams and tables)
- NLPHL has residual IgD positive mantle cells and CD21+ follicular dendritic cell meshwork (not present in THRLBCL)
- Neoplastic B cells:
- Peripheral T cell lymphoma such as angioimmunoblastic T cell lymphoma (large B cells are nonneoplastic): prominent infiltration of eosinophils, plasma cells and increased vascularity; CD10+ in T cells (TFH cells)
- Reactive conditions (drugs, viral infection, autoimmune diseases): preserved architecture; heterogeneous cytological appearance of immunoblasts; CD15- immunoblasts; EBER+ in both large and small lymphocytes (infectious mononucleosis)
- Relationship between T cell / histiocyte rich B cell lymphoma (THRLBCL) and nodular lymphocyte predominant Hodgkin lymphoma (NLPHL):
- Classical NLPHL has indolent clinical course, while THRLBCL is an advance stage, aggressive disease
- Cases of NLPHL are known to have morphologically transformed into THRLBCL
- There are cases of NLPHL with diffuse T cell rich, "THRLBCL-like" pattern which behaves less aggressively than de novo THRLBCL
- Evidence suggests these two entities may represent different spectrum of the same pathobiology
- Evidence suggesting similarity between both diseases:
- Neoplastic B cells show similar immunophenotypical profile
- Gene expression profiling (GEP) shows no clearly distinct gene expression pattern between the two diseases (PLoS One 2013;8:e78812)
- Comparative genomic hybridization (CGH) shows similar unique aberrations (REL locus) (Br J Haematol 2015;169:415)
- Evidence suggesting tumor microenvironment may affect manifestation of the disease:
- CD4+ T cells less frequent in THRLBCL than NLPHL
- CD163+ histiocytes more frequent in THRLBCL than NLPHL (Haematologica 2010;95:440)
- Evidence suggesting similarity between both diseases:
Additional references
Board review style question #1
Which one of the following statements regarding T cell / histiocyte rich large B cell lymphoma (TCHRLBCL) is FALSE?
- Differential diagnosis of TCHRLBCL include viral infection, peripheral T cell lymphoma and classic Hodgkin lymphoma
- TCHRLBCL and NLPHL show different clinical presentation and organ involvement
- The large cell component of both TCHRLBCL and NLPHL are CD20+, OCT2+, BCL6+ and EBV+
- The peritumoral environment of TCHRLBCL contains abundant CD8+ / TIA1+ cells
- 35 year old man presents with cervical and supraclavicular lymphadenopathy. Core biopsy shows scattered LP ("popcorn") cells in a background mostly of T lymphocytes and histiocytes and scarce B lymphocytes. The diagnosis is "nodular lymphocyte predominant Hodgkin lymphoma with T cell / histiocyte rich large B cell lymphoma-like pattern"
Board review style answer #1
C. is false: the large B cells are generally EBV negative
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