Diffuse large B cell lymphoma – T cell / histiocyte rich (THRLBCL)

Author:Konrad Chan, M.D.Senior Author:Rumina Musani, M.D.

PubMed Search:T cell / histiocyte rich large B cell lymphoma

AdvertisementPage views in 2018:5,690Page views in 2019 to date:2,283Table of ContentsCite this page:Chan K. T cell / histiocyte rich diffuse large B cell lymphoma. m website. Accessed May 13th, 2019.Definition / generalDiffuse large B cell lymphoma subtype with abundant nonneoplastic T cells and histiocytes, that may obscure tumor cellsEssential featuresMust 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 THRLBCLCaution when diagnosing THRLBCL in a small biopsy: if there is history of NLPHL, describe as THRLBCL-like transformation of NLPHLEvidence exists that NLPHL and THRLBCL represent different spectrum of the same pathobiological entityDiagrams / tables

Clinical featuresRare morphological variant of diffuse large B cell lymphoma (DLBCL); 1 – 3 of all B cell lymphomasUsually middle age adults, often with advanced stage (Ann Arbor III / IV) and involvement of lymph nodes, spleen, bone marrowClinical presentation: fever, malaise, splenomegaly or hepatomegalySame treatment protocol as DLBCL, NOS but lower complete remission rateCase reports32 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 of the Week 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;15:1051free full text)THRLBCL presenting on the skin (Dermatology 2011;222:225)Gross images

37 year old manMicroscopic (histologic) descriptionDiffuse or vaguely nodular patternScattered single neoplastic large B cells ( 10 of all cells) amongst small reactive T cells and histiocytesNear absence of B lymphocytes in the background; no remnants of B follicles or clusters of small B lymphocytesDiverse 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 granulomasCentroblast-like: pale eosinophilic cytoplasm, oval / round nuclei with vesicular chromatin and without nuclear atypia, small basophilic nucleoli adjacent to nuclear membraneHRS-like (Hodgkin Reed-Sternberg cell-like): large multinucleated cells with abundant amphophilic cytoplasm, pleomorphic nuclei with prominent eosinophilic or amphophilic round / central nucleoliNeoplastic B cells are surrounded by clusters of bland nonepithelioid histiocytesMicroscopic (histologic) images

Contributed by Drs. Asmaa Gaber Abdou and Nancy Youssef Asaad

CD20Positive stainsLarge B cells:Pan B markers and transcription factors (CD20CD79aPAX5, Oct2 /BOB.1)BCL6(50 – 91),EMA(~30, associated with lymphocyte rich/LR-like cells),BCL2(40)Rarely positive forCD30(8 – 30, usually stains HRS-like cells) andCD10(10)Reactive T cells:CD3+,CD5+,CD8+,TIA1+ (cytotoxic T cells)Reactive histiocytes:CD68+,CD163+Negative stainsLarge B cells:CD15, CD30,CD138, IgDBackground small lymphocytes: IgD (mantle cell marker)No FDC meshwork (CD21CD23CD35)(Rarely) T cell rosetting around tumor cellsEBV(otherwise defined as EBV+ DLBCL)Molecular / cytogenetics descriptionPAX5/IgHgene rearrangement by FISHDifferential diagnosisClassic 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 THRLBCLIgD+ favors NLPHLDifferent microenvironments (see diagrams and tables)NLPHL has residual IgD positive mantle cells and CD21+ follicular dendritic cell meshwork (not present in THRLBCL)Peripheral T cell lymphoma such asangioimmunoblastic 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 diseaseCases of NLPHL are known to have morphologically transformed into THRLBCLThere are cases of NLPHL with diffuse T cell rich, THRLBCL-like pattern which behaves less aggressively than de novo THRLBCLEvidence suggests these two entities may represent different spectrum of the same pathobiologyEvidence suggesting similarity between both diseases:Neoplastic B cells show similar immunophenotypical profileGene 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 NLPHLCD163+ histiocytes more frequent in THRLBCL than NLPHL (Haematologica 2010;95:440)Additional referencesJ Clin Pathol 2017;70:187Adv Anat Pathol 2016;23:202Mod Pathol 2013;26:S57Arch Pathol Lab Med 2010;134:1434Board review question 1Which one of the following statements regarding T cell / histiocyte rich large B cell lymphoma (TCHRLBCL) isFALSE?

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

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