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Richter, but nor CHL, nor DLBCL: NEW category by WHO? (754255)
Richter, but nor CHL, nor DLBCL: NEW category by WHO?new
Subtitle: B16-20877
Type:
HEMATO
Sender:
ugnius
2016-06-18 10:26
INCTR - EBMWG Hematopathology Online
YOU ARE WELLCOME!  
 
67 yrs old lady with long story of B CLL/SLL (in 2013 threpine with 30% invoilvement: NOT INCLUDED here).  
Lymph node biopsy was performed (1.2cm; location ND).  
 
HISTO: Diffuse and vaguelly nodular architecture, reminescent of "accelerated" B CLL. Interstitial multiple "immunoblastoid" and giant cells with EBER+ CD30+ Pax5+ faint CD15(-) BOB1/Oct2+ attenuated B programme (CHL like).  
Context: CD23+ CD5+ small medium B CLL/SLL population.  
 
PROPOSED DIAGNOSIS: B Aggressive lymphoma/Richter's (B CLL/SLL) transformation in the context of B CLL/SLL (accelerated/progression phase).  
QUESTION: a DEFINITE name of this Richter;s: "T cell rich B cell lymphoma EBV+, CHL like"? Or maybe Accelerated B CLL/SLL with EBER+ cells and high Ki67 index?  
 
"To West (Basel)" :).
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tzankov
2016-06-20 06:54
Hi Ugnius,  
 
nice case. In my eyes morphologic criteria for Richter's are not met. I would suggest calling this case paraimmunoblast-rich CLL/SLL with sacttered EBV+ large cells (these EBV+ cells are more the result of overmimmunosuppression due to the CLL or due to fludara and not a true sign of transformation; such patients may considerably benefit from immediate rituximab to prevent EBV propagation). The elevated amount od paraimmunoblasts and the higher proliferative acitivity of the CLL fit with the long clinical history and are indeed a sign of morphologic progression (but still not of Richter's tranformation).  
 
Alex
ugnius
2016-06-22 14:50
Thank you. Comparing with definitelly "accelerated" or "paraimunoblastic progression" cases this one shows a little bit more large blasts (like in "TcR LBCL EBV+"), but for sure nor DLBCL, nor CHL... And- you are true- EBER+ large cells are still scattered...
Last modified: 2016-06-18 10:39:34