Traumatic Subdural Effusion Evolving into Chronic Subdural ...
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10/11/2014
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Dr Stefan Dojcinov
University Hospital of Wales, Cardiff
Edinburgh Haematopathology Tutorial 2013 Cases 3 and 6
Case 3
Female, 83
Headaches and unsteadiness for 3 weeks. Found to have obstructive hydrocephalus due to a right cerebellar lesion.
Posterior fossa craniotomy and excision of right cerebellar lesion.
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CD20 CD3 PAX5
CD30 CD15 Oct2
EBER LMP1
Case 6
Male, 50 Shortness of breath, drenching night sweats and weight loss in November 2009. In early 2010 bilateral mid and lower zone lung masses, liver lesions and a (L) adrenal lesion thought to be non small cell lung cancer. Bronchial cytology not diagnostic. A bronchial biopsy showed necrosis with no malignancy. Treated empirically for NSCLC with gemcitabine and carboplatin, stopped in April 2010 but lung lesions soon after relapsed. Open biopsy left lower lobe lesion.
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PAX5 Oct2
EBER LMP1
Cases 3 2003 – Classical Hodgkin Lymphoma (EBV+)
2010 - EBV+ Age Related Large B-cell Lymphoma (LyG – like features)
Cases 6
Lymphomatoid Granulomatosis – Grad 3
Lymphomatoid Granulomatosis
Extranodal EBV assciated B-cell LPD
Angiocentricity and angiodestruction
Immunological deficit
CD8 dysfunction
Association with ID (WA, HIV, HTLV1, Transplant)
Age 30s-40s M:F=2:1 Western population
Lung Skin Liver Kidney Brain No lymphadenopathy No bone marrow involvement
LyG – Epidemiology & Presentation
AWLP® LyG – Pathological features
Polymorphous angiocentric infiltrate
– Lymphocytes
– Plasma cells
– Variable immunoblasts and HRS-like cells depending on grade
Copious necrosis
NO GRANULOMAS
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CD20 AWLP ®
AWLP ®
CD20 PAX5 CD3
CD30 EBER CD15
LMP1
LyG - Grading
Grade 1 <5 EBER+ cells / HPF
No atypia
Grade 2 5-20 EBER+ cells / HPF
Occasional large cells
Small clusters
Grade 3 Numerous EBER+ large cells
No diffuse infiltrate
Case 3 Case 6
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Angioinvasion in EBV+ LP
Necrosis and angioinvasion in NK/T-cell and LyG
EBV capacity to induce monokines
Interferon-g–inducible protein-10 (IP-10)
Monokine induced by interferon-g (Mig)
Teruya-Feldstein et al. Blood, 1997; 90: 4099-4105
Age Related EBV+ LP (AREBVLP) or LyG
AREBVLP LyG
Presentation Nodal and extranodal Extranodal No diagnosis without lung involvement
Morphology Range of cell sizes Histiocytes, lymphocytes and PCs Angioinvasion Occasional granulmas Necrosis Numerous EBER+ cells (variable size)
Range of cell sizes Mainly lymphocytes Angioinvasion No granulomas Necrosis Numerous EBER+ cells (variable size)
Phenotype CD45+/- CD20 +/- PAX5+ OCT2+ BOB1+ CD30+ CD15+/- (68%) LMP1+
CD45+ CD20+ PAX5+ OCT2+ BOB1+ CD30+ CD15- LMP1-
Case 3 - Follow up
Extensive chest infection post op
Poor performance status
30G radiotherapy in April 2003
Died 3 months later with multiple spontaneous subdural haematomas and probable disease relapse
Case 6 - Follow up
Bone marrow negative, no lymphadenopathy
Received 8 courses of R-CHOP between November 2010 and June 2011
CT post 5 cycles showed a marked reduction in all lesions;
PET post 8 cycles showed no definite tumour
No further chemotherapy and remains in remission
Chest X-Ray is currently clear
LyG – Treatment and Prognosis
No standard protocols
Grades 1-2
Interferon alpha2b
Grade 3
Chemotherapy (R-CHOP)
Variable outcomes but median survival 14 months