69 yrs male with retroauricular lymphadenopathy.  
SONO: Hepatomegaly. Blood values: normal, except slight eleveation of CRB, ESR and eosinophilia (FILE IS ATTACHED).  
Lymph node biopsy was taken.  
HISTO: "Marginal" or interfollicular lymphoproliferation with "pseudo-low grade" cytology: oval- irregular pleomorphic nuclei with some "blastic" appearance" and high proliferative activity. Vascularisation CD34+ is minimal. NASDE(-) clear minimal cytoplasm.  
IH: CD3/CD2(++/+++) 100%, CD4(++/+++) 100%, CD5(+) 60% (weak), CD7/CD8(-), CD79a(-), Bcl6(++/+++) 100%, CyclinD1/CD23(-), CD20(+/++) 30% , TdT(-), CD34(-)(minimal vascular network), CD99(+/+++) 40%, CD117(-)(mastocytes +), EBV LMP1(-), CD1a(-)(focal Langerhans cell hyperplasia), CD15/MPO/CD30/ALK1(-), CD21/CD23(-). Residual CD21/CD23+ FDC networks in follicles. Ki67 prolif. activity HIGH ~70%.  
 
Cytotoxic molecules and T/B clonality pending.  
 
WORKING: High grade lymphoma with T phenotype (Peripheral T lymphoma NOS: CD3/CD4/CD2+; CD5+/-; CD7-) and aberant B (CD20; Pax5) and stem cell markers (CD99). DIFFERENTIAL: "blastic" (myeloid) leukaemia/lymphoma.  
 
Thank you for participation and support.

Mueller-Hermelink 2008-12-07 19:00	A nice and interesting case! The basic histological pattern is that of a T zone lymphoma of the Kiel Classification: Interfollicular infiltration , clear cell character of tumor cells , mostly "lymphocytic" appearance but high proliferation rate , CD4 positivity .Some of these cases are now in the follicular variant of PTCL-NOS with a phenotype of follicular TH-cells ( PD1 +, Icos+ ) which is also suggested by BCL6 positivity some coexpress CD10 and CD30 ( weak).Nonfitting are the the equivocal ( in my view) reactivities for CD20 and Pax5 . I am not sure whether the staining is really in the tumorcells and if overstaining might be a problem - at least I would not overinterpret these findings. Some of these cases in our experience were focal ( stage I or II) but some experienced recurrences indistinguishable from AILT.   In conclusion ,I would favour a PTCL-NOS , follicular variant.
ugnius 2008-12-07 20:38	Dear Prof.Hermelink,     Really nice to hear from you in Late Sunday:) Thank you for a urgent comment. Our case is CD10-CD30-, PD1 is unfortunatelly absent still. At least I‘ve added CD56/57 and cytotoxic panel for full documentation. I will be back with new stains. It's true about CD20+, but PAX5 is uniformly positive like Bcl6.
Mueller-Hermelink 2008-12-08 15:19	Dear Dr. Ugnius! The case is fascinating ,- but there is still a question whether there is a co-expression in the tumor cells or a mixture of different cell populations, I would do double and triple stainings to clarify this point ( and offer to do that ,if you send me a block). On the other hand I truely think that this would be more of acaqdemic interest , since it probably would not change the diagnosis.
ugnius 2008-12-08 15:44	At least I 've appended some virtual slides with Pax5, CD20, CD3 and HE. Unfortunatelly the block may be exhausted after add stains and molecs. I will be back with add photos and PCR results. Double stains I hope will be once more introduced in our center in weeks after some break and I will do it in future, but more probably on ither cases. Please respond if You cannot open the virtual slide links.
bvrugt 2008-12-09 10:42	I also find the IHC results from the T-cell markers more convincing than the immunoreactivity for e.g. CD20, and therefore favor a peripheral T-cell lymphoma NOS. However, I agree with you that the Pax-5 expression is curious. A little search on PubMed lead me to a manuscript from our collegue Tzankow: Rare expression of BSAP (PAX-5) in mature T-cell lymphomas. Mod Pathol. 2007 Jun;20(6):632-7.
tzankov 2008-12-11 10:59	I could not open the virtual slides. I agree with all previous comments. Consdiering B-cell markers in T-NHL, there is an interesting paper in one of the latest AJSP issues. Our own observations, already mentioned above, and some experiance by Prof. Pileri (personal communication) show that abot 1% of PTCL, NOS express PAX5.     Rahemtullah A, Longtine JA, Harris NL, Dorn M, Zembowicz A, Quintanilla-Fend L, Preffer FI, Ferry JA. CD20+ T-cell lymphoma: clinicopathologic analysis of 9 cases and a review of the   literature. Am J Surg Pathol. 2008 Nov;32(11):1593-607.     PMID: {18753947}
ugnius 2008-12-12 10:08	Appologies for temporary technological troubles: we are just learning IT:) PLease check once more the links to virtual slides and reply (printscreen) if any troubles persist. Thank you for patience.
hurwitz 2009-01-01 21:32	I have nothing to add to the previous comments. I also agree with the diagnosis peripheral T-cell lymphoma NOS, T-zone variant.   Thanks for the reference on CD20 positivity in T-cell lymphomas.   Did you receive the results of TCR clonality?   PS, I managed to open the virtual slides, but they can be viewed on low power only. Trying to enlarge, the image gets completely blurred.
ugnius 2009-01-02 10:47	MOLEC'S: TCR beta CLONAL, TCR gama and IgH/KAPPA polyclonal. Thank you for all done.
hurwitz 2009-02-09 17:10	Having the results of PCR proofing TCRB clonality, the diagnosis of PTCL, NOS (T-zone variant) is established.   The most interesting feature of this tumor is the aberrant expression of CD20. Thanks to DR.Tzankov for the reference.   Thanks also to Ugnius for letting us share this fascinating case.
kunze 2009-02-10 16:33	A note to the virtual slides: I can open the VS up to the highest magnification with my home PC but not with a PC in my past institute and I feel some colleagues have the same problem. Apparently, the configuration of some firewalls is not compatible and the user of iPath need a generally applicable solution.
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