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Does CHL lymphocyte depleted variant exist in 2018? (945009)
Does CHL lymphocyte depleted variant exist in 2018?new
Subtitle: B18-4420
Type:
HEMATO
Sender:
ugnius
2018-02-17 16:07
INCTR - EBMWG Hematopathology Online
STORY: 19 yrs male with neck/axillary and mediastinum lymphadenopathy and fever with night sweats, hepatomegaly, ascites. Lymph node blocks (axilar) was sent for second opinion with diagnosis of Hodkin's lymphoma. Bone marrow biopsy: NEGATIVE.  
 
HISTO: Solid diffuse sheets of medium large cells with scattered multinucleated or lobated giant cells ("DLBCL or ALCL like pattern") with purelly CHL immunophenotype: EBER(-); CD30+ > CD15+; LCA(-); Fascin+; CD21(+/-); Mum1+; CD3/CD2/CD5+ (focal abberant); ALK1(-); BOB1/Oct2(-); Pax5+ (weak); CD20/CD79a/Ig kappa/lambda/CD138(-); Bcl6/CD10/CyclinD1(-); EBV LMP1(-); EMA(-); CD4/CD7/CD8/GranzymB(-). Necrosis up to 30%.  
MOLECS: B clone: IGH locci FR1, FR2 and D-J.  
 
Follow up: escBEACOPP scheme applied. Lymphadenopathy was reduced up to 30%, no fever, organomegalies or other complications are absent too. No trasfussions at the moment.  
 
 
PROPOSAL: Still classic Hodgkin's lymphoma, but: SINCYTIAL NS2 or just LYMPHOCYTE depleted (I'm very sceptic about that...). Some T Ag expression as worse prognostic sign?
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tzankov
2018-02-19 18:51
Well, you falsified the DD of ALK- ALCL (you may additionally stain the case for p63 (+ in some ALCL, - in cHL) and for PDL1; by means of that and the B-cell clonality this is a bona fide cHL, unclassifiable subtype, more probably NS2 (apply polarized light; doing that you may see birefringent collagen that is more typical of NS).  
 
T-cell marker expression in cHL is well documented and usually prognosticall unfavorable (Blood. 2013 Mar 7;121(10):1795-804. doi: 10.1182/blood-2012-06-439455. Epub 2013 Jan 10.)
ugnius
2018-02-20 09:18
Thanx. I'll append info about PDL1 and p63. By the way: what clone and for different purposes You use in Basel? we have there: PD-L1(SP142) and PD-L1(SP263). Only rarely use because no medications available in LT.
tzankov
2018-02-20 17:25
for diagnostic purposes I use E1L3N (cheaper and robust)  
 
Evaluation of the diagnostic and prognostic value of PDL1 expression in Hodgkin and B-cell lymphomas. Menter T, Bodmer-Haecki A, Dirnhofer S, Tzankov A. Hum Pathol. 2016 Aug;54:17-24.
Last modified: 2018-02-19 12:50:28