EQA SCHEME CIRCULATION 33 EDUCATIONAL SLIDES DR GRAEME SMITH MONKLANDS DGH
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1 EQA SCHEME CIRCULATION 33 EDUCATIONAL SLIDES DR GRAEME SMITH MONKLANDS DGH
2 CASE E1 M: 68 yrs Left destructive sinonasal lesion.?lymphoma?adenocarcinoma
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6 CD20
7 CD10
8 BCL6
9 MIB1
10 Answers Diffuse large B cell lymphoma 47 Burkitt Lymphoma 13 Diffuse large B cell lymphoma, Burkitt-like 12 B cell lymphoma unclassifiable with features intermediate between DLBCL and Burkitt Lymphoma 7 Diffuse large B cell lymphoma vs Burkitt lymphoma - 5
11 Answers Lymphoma 3 NK cell lymphoma 2 Lymphomatoid granulomatosis 2 High grade lymphoma 1 Lymphoepithelial carcinoma (EBV driven) - 1
12 Further tests ISH for EBV Negative FISH shows t(8;14) C-myc C translocation FISH for t(14;18) negative Best regarded as B-cell B lymphoma, unclassifiable, with features intermediate between DLBCL and Burkitt lymphoma
13 CASE E2 F: 82 yrs. Weight loss, anorexia. Massive lymphadenopathy either side of diaphragm. CT scan suggests lymphoma.
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17 CD30
18 CD15
19 CD20
20 CD79a
21 MUM1
22 LCA
23 Answers Classical Hodgkins Lymphoma (varying subtypes) 43 Diffuse large B cell lymphoma (some?anaplastic variant) 26 B cell lymphoma, unclassifiable, with features intermediate between DLBCL and Classical Hodgkins 6
24 Answers Anaplastic large cell lymphoma 5 Diffuse large B cell lymphoma of the elderly -3 Nodular lymphocyte predominant Hodgkins 3 Hodgkinoid large B cell lymphoma 2 Lymphoma 2 Diffuse large B cell lymphoma vs Nodular lymphocyte predominant Hodgkins 1 Hodgkins vs Grey Zone lymphoma - 1
25 Further tests Positive immuno PAX-5, OCT2 and BCL- 6(weak) Negative immuno EMA, AEK-1, BOB-1 ISH for EBV negative No light chain restriction Best regarded as B cell lymphoma, unclassifiable, with features intermediate between DLBCL and Classical Hodgkins Lymphoma?Role for Retuximab given strong CD20 positivity
26 Borderline Category Lymphomas September 2008 WHO Classification Introduction of provisional borderline categories Overlapping clinical, morphological and/or immunophenotype/cytogenetics
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28 Intermediate DLBCL/BL DLBCL pathogenetic pathway deregulation of B-cell B signaling or transcriptional regualtion Target genes/molecular pathways BCL6 gene rearrangement, NFkB activation, STAT6 activation, BCL2 overexpression, MYC overexpression
29 Intermediate DLBCL/BL BL Pathogenetic pathway deregulation of cell cycle Target genes/molecular pathways EBV, MYC/IGH rearrangement
30 Intermediate DLBCL/BL Histology DLBCL Large cells prominent nucleoli Fewer mitoses Apoptosis and starry sky less common BL Medium sized cells, multiple nucleoli Mitoses Apoptosis and starry sky
31 Intermediate DLBCL/BL Intermediate DLBCL/BL Medium sized cells resembling BL Admixed large cells with prominent single nucleoli and nuclear irregularity High mitotic rate Apoptosis and starry sky pattern
32 Intermediate DLBCL/BL DLBCL/BL Immuno B cell antigens CD19, CD20, CD22, CD79a CD10, BCL6 BCL2 varying positivity MUM 1 negative
33 Intermediate DLBCL/BL MYC-IG translocation can be absent or complex with additional rearrangements of BCL2 and/or BCL6 (double/triple hit) Highly aggressive behaviour
34 Intermediate DLBCL/BL May have history of follicular lymphoma Present with generalised lymphadenopathy, extranodal masses, bone marrow involvement Heterogenous group Double/triple hit respond poorly to treatment (BL regimen/chop) Others treated with BL regimen Generally poor outcome Classification important for research
35 Intermediate DLBCL/cHL Previously called gray zone lymphomas Young men Highly aggressive Anterior mediastinum
36 Intermediate DLBCL/cHL Single tumour can have areas resembling chl, DLBCL or PMLBCL Inflammatory infiltrate usually sparse Necrosis Overlapping immunophenotype/gene expression between DLBCL, chl & PMLBCL
37 Intermediate DLBCL/cHL CD45 + B cell antigens (CD20, CD79a, OCT2, BOB-1, Pax5 CD10 negative ALK absent Co-expression of above markers with CD15 and/or CD30
38 Intermediate DLBCL/cHL No consensus on optimal treatment? Treat as aggressive high grade lymphoma? Role for addition of retuximab Carbone et al. Human Pathology (2010) 41,
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