17 yo girl with history of progressing cervical lymphadenopathy since late March. No B-symptoms until the last week, when she noticed fever and minimal weight loss. Imaging - bulky cervical and mediastinal process, no other sites involved. Mild neutrophil leukocytosis (WBC 13.000, nf 79%), PLT 650.000, mild iron deficiency, LDH and CRP only slightly elevated.  
Supraclavicular lymph node biopsy showed sheets of large to giant very pleomorphic cells, stellated necroses, only occasional collagen bands.  
Phenotype: LCA negative/weak, CD30+, CD15+, CD3-, ALK1-, CD20+, CD79a-, PAX5 negative/weak, MUM1+, CD138 partly+, EMA weakly+ (sorry, no pic!), bcl2+, CD10-, bcl6-, EBV LMP1- (I have no other options), CK AE1/3-, Ki67 about 30%.  
No infiltration on marrow cytology and trephine.  
The question: does it fit the gray zone (between DLBCL and cHL) or just cHL?  
A by-thought: bcl2 is bad for HL, could it be the same here?  
 
Many thanks for assistance!  
 
P.S. Meanwhile, the girl turned 18 and was transferred to the adult hospital with all biopsy material.

vrugt 2011-05-16 16:12	The morphology alone with sheets of tumor cells and sclerosis (image 8) suggests a classical Hodgkin with a DLBCL and an anaplastic large cell lymphoma as the main diff. diagnostic possibilities. However, the phenotype with expression for both CD30 and CD15 is quite characteristic for a classsical Hodgkin, and also CD20 and Pax5 are frequently expressed by Hodgin cells. I don`t think there is enough evidence for a grey zone lymphoma or DLBCL.
tzankov 2011-05-17 14:58	I can only support what has been said: classical Hodgkin lymphoma with very impressive Hodgkin and Reed-Sternberg cells. Ki-67 is low, in my opinion it is higher in classical Hodgkin lymphoma. I think it fulfills the criteria of NS2. A coexpression of CD20 and CD138 is more commonly observable in HL in immunosuppressed individuals, as is lower Ki-67. Is the patien immunosuppressed? HIV? Other?
SergeyN 2011-05-18 10:12	Dear Alexander, no immunosuppression, no HIV.   There are two things that make me uncomfortable: first, actually all tumor cells are clustered in bands; second, unusual phenotypic features like CD138, low Ki67 and (sorry, I've forgotten to upload) weak EMA.   Clinical presentation with asymptomatic lymph node enlargement and minimal inflammation background is not very typical, too.
tzankov 2011-05-18 12:47	I see..., but the cells have the typical book-cover morphology of Hodgkin and Reed-Sternberg cells, express CD30 and CD15 and are CD45-negative; she has a mediastinal mass! There are actually no realistic differentials.
diane.c.farhi 2011-05-18 19:02	I agree with the diagnosis of classical Hodgkin lymphoma with a very high population of RS and Hodgkin cells.
SergeyN 2011-05-19 10:47	Well, that means the question about bcl2 has been cleared as well.     I've got another question, about CD138. After Dr. Tzankov's comment I had a look at the Blue Book, and it was very strict on the matter: "...CD138 is consistently absent..." (p.328) and refers to the article by Buettner at al (http://onlinelibrary.wiley.com/doi/10.1002/hon.764/pdf). Still, there had been only 43 cases studied, too few by far to make absolute rules.     So there are exceptions, aren't there?
tzankov 2011-05-19 13:59	Look at:     1: Carbone A, Spina M, Gloghini A, Tirelli U. Classical Hodgkin's lymphoma   arising in different host's conditions: pathobiology parameters, therapeutic   options, and outcome. Am J Hematol. 2011 Feb;86(2):170-9. doi: 10.1002/ajh.21910.   Review. PubMed PMID: 21264899.     2: Tzankov A, Zimpfer A, Pehrs AC, Lugli A, Went P, Maurer R, Pileri S, Dirnhofer   S. Expression of B-cell markers in classical hodgkin lymphoma: a tissue   microarray analysis of 330 cases. Mod Pathol. 2003 Nov;16(11):1141-7. PubMed   PMID: 14614054.     3: Watanabe K, Yamashita Y, Nakayama A, Hasegawa Y, Kojima H, Nagasawa T, Mori N.   Varied B-cell immunophenotypes of Hodgkin/Reed-Sternberg cells in classic   Hodgkin's disease. Histopathology. 2000 Apr;36(4):353-61. PubMed PMID: 10759950.
SergeyN 2011-05-25 12:14	Dear Dr.Tzankov,     Thank You!!!!
SergeyN 2011-07-08 14:23	Dear friends,   I am very grateful to all participants for clearing up the case.
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