Advances in Hematology / 2012 / Article / Tab 1

Review Article

Grey Zone Lymphomas: Lymphomas with Intermediate Features

Table 1

Common and distinguishing features of PMBCL, cHL-Nodular sclerosis (NS), and B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and cHL (BCLu-DLBCL/cHL). Modified after Hasserjian et al. [23].

PMBCLcHL-NSBCLu-DLBCL/cHL

Common features
 AgeYoung patientsYoung patientsYoung patients
 GenderFemale predominanceFemale predominanceMale predominance
 LocalizationMediastinal mass eventually supraclavicular lymph nodesMediastinal mass eventually supraclavicular lymph nodesMediastinal mass eventually supraclavicular lymph nodes or more rarely other lymph nodes
 MorphologyCompartmentalizing fibrosisFibrosis in thick bandsConfluent, sheet like growth of pleomorphic tumor cells with diffuse fibrotic stroma Variability from area to area
 Therapy responseRadiotherapy sensitiveRadiotherapy sensitive
 ImmunophenotypeLack of Ig-Expression
Lack of HLA I expression
Frequent CD30 expression
Expression of MAL and CD23
Lack of Ig-expression
Lack of HLA I expression
CD30 expression
Transitional features between PMBCL and cHL
B-cell program often preserved
 Genetic and  molcular featuresExpression of HLA-I
REL (2p15) and JAK2 gains (9p24)
CIITA breaks
Activation: NF-kappaB, JAK-STAT (incl. STAT6), and P13K/AKT pathway High expression of extracellular matrix elements, overexpression of TNF family members
Expression of HLA-I
REL (2p15) and JAK2 gains (9p24)
CIITA breaks
Activation: NF-kappaB, JAK-STAT (incl. STAT6), and P13K/AKT pathway
High expression of extracellular matrix elements, overexpression of TNF family members
REL (2p15) and JAK2 gains (9p24)
CIITA breaks

Distinguishing features
 MorphologyClear cells often homogenous (but Reed Sternberg cells may occur)
Little or no inflammatory background
Hodgkin cells and Reed Sternberg cells
Typical inflammatory background
 ImmunophenotypeB-cell markers preserved (CD20, CD79a, PAX5) B-cell transcription factors present (BOB.1 and OCT-2)
CD15 absent
Absence of EBV
B-cell markers lacking or only weakly or heterogeneously expressed (especially PAX5) B-cell transcription factors usually negative
CD15 may be present
EBV may be present