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UNUSUAL MONOMORPHIC CHL NS2 CD21+ (577077)
UNUSUAL MONOMORPHIC CHL NS2 CD21+new
Subtitle: B14-22913
Type:
HEMATO
Sender:
ugnius
2014-06-27 07:31
INCTR - EBMWG Hematopathology Online
83 yrs old male with previous core biopsy (inguinal node): "Aggressive B LPD with IH: PAX5/MUM1/CD30+, BCL6+(40%), CD21+, LCA+/-, OCT2(+/-), BOB1(+/-), KI67: 90%, CD20/CD79A(-), CD15(-), CD138/KAPPA/LAMBDA(-), ALK1(-), CD22(-), CD3/CD2/CD5/CD7/GRANZYMB(-), EBER_CISH(-).  
DLBCL with aberrant IH probable".  
 
WHOLE node biopsy was taken.  
HISTO: Nodular architecture with some septation. Nodules with peripheral rim of T lymphos, second inner CD68+ Ma rim and central nodular part formed by large mononuclear cells with necrosis and admixture of eo's.  
IH: the same. More clearly visible, that tumor cells Pax5+; CD20/CD79a/Oct2(-); EBER/LMP1(-); BOB1+ only singles; LCA(-)(except outer rim of macrophages, creating BINARY architecture of the nodules.  
MOLECS: IGH ir IGK polyclonal. TCRB V-D-J1/2 possibly clonal in the polyclonal background. TCRG polyclonal.  
 
INTERESTING FEATURE: aberrant CD21 positivity (described before:  
Kim SH1, Choe JY, Jeon Y et al. Frequent expression of follicular dendritic cell markers in Hodgkin lymphoma and anaplastic large cell lymphoma. J Clin Pathol. 2013 Jul;66(7):589-96. doi: 10.1136/jclinpath-2012-201425).  
 
PROPOSAL: CHL NS grade 2 (sintycial)?  
 
Thank you for continous support.
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tzankov
2014-06-27 17:24
I miss CD15 and EBER on the "WHOLE node biopsy".  
From what I see an age-related DLBCL (Hodgkin-like) must be excluded.  
BCL6 and such a strong PAX5+ are not typical of HL.  
The cytomorphology is not typical for HL too.
ugnius
2014-06-27 20:16
Well, in biopsy I was thinking about so called "sinusoid" DLBCL. But immunophenotype is very truncated. Pax5 is strong, true. But IH still close to CHL. I usually do not post IH negatives, but will do that next week or tommorrow, if scanned. It seems "grayish", don't you?
ugnius
2014-07-01 08:19
Please find EBER (negative, plasma cell cytoplasmic noise only) and CD15 (negative, only necrotic area).
tzankov
2014-07-01 11:32
classificatory difficult case. I am aware of such cases only in immunosppressed individuals, where the cathegory of Hodgkin-like aggressive lymphomas has been created (PTLD and iatrogenic immunosppression-associated LPD; is the patient immunosuppressed MTX? Steroids? TNF-anatagonists? Colhicin?).  
 
the morphological appearance is that of a large cell, immunoblastic B-cell lymphoma. the phenotype is equivocal, but does not preclude a DLBLC (especially post-GC/preplasmablastic B-cells can show similar phenotypes, e.g. solid variants of PEL). EBER-negtaivity rules out a Hodgkin-like DLBCL of the elderly.  
 
the age, the morphology, the negativity for CD15, the expression of BCL6, BOB1 and the intensity of PAX5 would rather force me thinking of unique DLBCL variants than of Hodgkin. HHV8 might be worth to be stained. a detailed clinical examination of any immunosuppression or local prostheses might bring more than any additional staining.  
 
it the patient immunocompromized?
ugnius
2014-07-01 11:53
Thanx,  
 
1. Hemato data: no other disease or known immunodeficiency.  
2. Its strange when we face with CD20 terminal loss and have no Ig or CD138 expression...  
3. At the moment I still not have HHV8, but it arrive in days- will be stained.  
4. WHICH IH you recoomend to implement for "plasmablastic" DifDx?  
 
Maybe "Aggressive B lymphoma with grey zone DLBCL/CHL features"? (please dont kill me for greyiness ever...)  
Thank you for all done. Best wishes.
tzankov
2014-07-01 12:06
you can add CD38 form point 4.
ugnius
2014-07-18 12:00
The last "makeup": PCR: IGH ir IGK polyclonal. TCRB V-D-J1/2 possibly clonal in the polyclonal background. TCRG polyclonal.  
Maybe too scant population? Maybe more close to cHL?  
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Last modified: 2014-07-18 11:59:47