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Classic Hodgkins Lymphoma, EBV+, hemophagocytosis (6746)
Classic Hodgkins Lymphoma, EBV+, hemophagocytosisclosed
Subtitle: B08-36490
Type:
lymph node
Sender:
ugnius
2009-01-15 10:03
INCTR - EBMWG Hematopathology Online
HISTORY: 41 yrs old male presents with B symptoms and hepatosplenomegaly only (CT not reveal any lymphadenopathy). Single subclavicular node was hardly found and biopsied as well as threpine was made.  
Blood: cytopenia (BM obliterated). BM: hemophagocytosis (original smear in the hospital) and giant cells.  
NODE: Classic RS and H cells in moderate cellular histiocytic background with multiple cytotox CD8+ cells with marked nuclear irregularities.  
BM: 80% of the population- diffuse involvement of the same tumor.  
IH NODE: Giant cells (cHL): CD30+; CD15+; CD20+/-; LCA+/-; EMA-; EBV LMP1+; Pax5+; CD79a/CD43(-).  
IH BM: Giant cells: CD30+; CD15+; CD20+; EBV LMP1+; CD79a-/+ (scattered); cyclinD1+.  
INFILTRATE: CD68+CD4+ Ma reach; CD3/CD8/Perforin/TIA1/GranB+ >> CD20+ lymphos with marked nuclear abnormalities of cytotox T cells.  
T population: CD3+ 100%; CD7/CD5/CD2+ 70%; CD8+ >> CD4+.  
PCR: gama/beta polyclonal background with possible "small" clone. B clonality in progress (laser microdissection technique is implemented).  
VIRTUAL SLIDES:  
HE: http://193.219.87.170:82/imageserver/LT_TELEPATHOLOGY/Ugnius/Studentams/B08-36490_Hodzkino_limfoma_misriu_lasteliu_variantas.svs/view.apml?  
 
BM: http://193.219.87.170:82/imageserver/LT_TELEPATHOLOGY/Ugnius/Studentams/B08-36486_Hodzkino_limfoma_misriu_lasteliu_variantas.svs/view.apml?  
 
PROPOSAL: cHL: MC (enreached in macrophages and cutotox T cells).  
QUESTION: 1. Nuclear irregularities in CD8+ CD3+ cytotoxic T population and diffuse (not focal as usual) involvement of bone marrow. 2. Some giant cells in lymph node seems to be LCA+ (majority) and in BM CD79a+ (focal, minority)
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tzankov
2009-01-16 11:02
for me it is a cHL, EBV+, MC. I think tht the HRSC are LCA-, and there is some staining bias from the accompanying background infiltrtate. would you add the CD79a pictures? thank you!
ugnius
2009-01-16 11:15
Thank you. CD79a is replaced.
tzankov
2009-01-16 18:24
Thanks for the CD79a staining. It is difficult to dtermine if this is a HRSC or a large B-blast, since I can not determine the nuclear details, but occasional CD79a-positivity of HRSC would be still compatible with cHL.
hurwitz
2009-01-18 11:24
This is a very interesting case. I agree that the morphology and immunophenotype are compatible with cHL. In addition there are features suggesting HL associated with immunedeficiency: EBV association, hemophagocytosis on the aspirate (unfortunately not shown), RS cells strongly CD20+ and primary extranodal presentation with massive BM involvement. Please try to find out if there is any history of RA treated with methotrexate (methotrexate associated LPD?) or other causes of immunedeficiency.
ugnius
2009-01-19 09:45
Ig levels in blood are normal. Any autoimmune diseases were present. Virusological studies are negative.
torlakovic
2009-01-19 17:00
To me this looks like cHL, EBV+. You have ruled out immunodeficiency-related disorder. Wide range of CD4:CD8 ratio (one of the most recent papers indicates range of 0.44-22) may be seen in the background and reactive/activated T-cells may have some degree of morphological variation including occasionally somewhat atypical appearance. Rare case of peripheral T-cell lymphoma associated with EBV+ RS cells have been mentioned in the literature (Am J Surg Pathol. 1999 Oct;23(10):1233-40). If you PCR test is highly sensitive, I would think that you ruled out such a possibility, too.
ugnius
2009-01-19 19:40
Thank you a lot.
hurwitz
2009-02-13 18:28
Please do regard Dr.Torlacovic's comment as the final one.  
Thank you.
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Last modified: 2009-01-15 10:03:07