37 yrs old male with history of T-LGL from 2001, treated non-systematically there and in Russia (after words without Mtx). In April 2009 he underwent surgery due to acute abdomen: multiple ileal perforations, abscesses and difusse peritonitis were found. Soon after operation the patient passed away. EBV evaluation was not done.  
HISTO HISTORY:  
2001: BM: Pleomorphic T proliferation and myeloid depression.  
2007: BM: T proliferation CD3/CD2/CD5+ CD7+/- CD8+ GranzymB-/+, T-LGL (or LGL lymphocytosis) probable. Plasmacytosis (politypical) 10%.  
2008: soft tissue abscess (not included in photos).  
2009: BM: T-LGL + prominent politypic plasmacytosis (LPD?).  
ALL BM CHANGES (review) similar/identical.  
 
HISTO INTESTINE: 1) Pleomorphic lymphoplasmacytoid infiltrates in ulcerations and serosa of small intestine and regional lymph nodes; 2) Scarse atypical CD8+ T infiltration in the intestine wall (esp subserosa/serosa) with some typhus like "granuloma" formation.  
IH: 1. PLASMA INFILTRATION: CD20(-); CD79a(-)(singles faintly +); Pax5(-); Mum1+; IG kappa/lambda+ (polytypic); EBV LMP1(-)(sporadic incidental +); Ki 67 very high.  
2. T population (interstitial in background of plasma-population, in muscularis and subserosa/serosa: CD3+; CD2+; CD7+ > CD5+; CD8+ >>> CD4+; CD57+; CD56(-); TIA1+ > GranzymB+ (in part); CD30/EBV LMP1(-).  
PCR: B polyclonal; T clonal (beta and gama).  
 
PROPOSAL: 1) Extranodal polymorphic B LPD, asociated with EBV in T-LGL/therapy induced immunosupression? 2) T-LGL in BM and minimal residual extranodal disease (clonal).  
 
SPECIAL QUEST: The rarity of constelation of these changes (extranodal T-LGL disease, B-LPD polymorphic after T-LGL, aggressive course of T-LGL)???  
Thank you for continous support.

tzankov 2009-12-18 14:21	Rather complicated. What I see is ulcerated pleomorphic lesion with plasmablasts. The proliferation is worrisome as well as the cytology. It is probably an immunosuppression/infection (EBV?)-associated lymphoproliferative disorder. I am not sure that the LMP1 staining is specific, since it stains cells that look like mature plasma cells. Are there any Hodgkin-like cells that stain for LMP1? Are there LMP1+ large blastic cells? Necroses? What about EBER? Would you please have a look for cytotoxic cells (CD57, TIA1, Granzyme B, CD3, CD8, CD4?).
hurwitz 2009-12-21 18:58	The histology looks quite like an IM like polymorphic PTLD. In this case chemotherapy is the most likely cause for the immuno-supporession There are numerous immunoblasts, but still the full range of plasma cell maturation can be seen. I share Dr.Tzankov's doubts about the specifity of the LMP1 stain.   It is important to find out if the patient is still receiving any chemotherapy, since it may regress after cessation of chemotherapy.   Will you be able to provide us images of the previous biopsies? What was the basis for the T-LGL diagnosis?
ugnius 2009-12-22 09:03	Thank you for early comments on hiden case:) I will append the rest of photos in minutes. Appologies for delay.
leoncini 2009-12-22 12:58	I am not sure that the LMP1 staining is specific, since it stains cells that look like mature plasma cells.   I am not sure if this lesion is a lymphoma
ugnius 2009-12-22 13:01	Please find all required data above and some virtual slides. Thank you.
tzankov 2009-12-30 07:02	Dear Ugnius, thank you for adding the bone marrow histology. There is little doubt on the presence of a T-cell lymphoproliferation consistent with LGL in the bone marrow. What about these highly hyperplastic plasma cels in the bone marrow, where they clonal or not, I suppose not? What about EBER in the intestinal lymphoproliferation?
ugnius 2009-12-30 07:55	Thanx a lot. BOTH plasmacytic huperplasias in the intestine and BM POLYTYPIC (and polyclonal in the intestine). EBV LMP1 really (-), I'm waiting for implementation of the new EBER procedure there: it will take some time.
torlakovic 2010-01-04 07:10	I agree that the bone marrow shows T-cell lymphocytosis consistent with LGL. I would review material from the time LGL diagnosis was first made to be sure that this is what it was. What about HIV status? In the slide labeled B09-12583_HE_9.svs there are large necrotizing granulomas and I wonder if you did histochemical stains like AFB and GMS?   Plasma cells have atypical morphology and very high proliferation rate and looks like this process is compatible with immunodeficiency related LPD. I would like to see both HHV8 and EBER.
hurwitz 2010-01-06 21:25	There seems to be little doubt on the diagnosis of T-LGL. The most interesting part of the case is the plasma cell hyperplasia seen in the intestinal tumor, the regional lymph node and the bone marrow. In all three lesions the plasma cells have an atypical morphology and are polyclonal. In addtion they form plasma cell nodules in the bone marrow, a feature usually seen only in immunodeffciencies. These changes are compatible with early lesions of PTLPD (plasma cell hyperplasia) as described in solid organ recipients. Which is essentially in agreement with Ugnius' suggestion. I cannot comment on the frequency of this constellation, but I guess it is very rare.   What about the serum globulins, are they elevated? The CD30+ cells in the lymph node may be an indirect indication for EBV. Let's wait for EBER.   Did you look for birefringent material in the subserosal granuloma?
raphael 2010-01-28 12:04	I share the comments from all the consultants   I have some doubts on a T-cell LGL proliferation and the clonality by PCR of T-cell can be observed in non tumoral process   I agree that the major feature of the lesion would be the plasmacytic infiltrate which is pleomorphic with some atypical plascytoid cells   The suggestion to know the HIV status is quite important, I have seen such aggressive lesions with large amount of polytipic plasma cells in some immunodeficient HIV+ patients   The presence of EBV must be checked by EBER   The detection of HHV8 must be also tested     atypical plasmocityc proliferation could be suggested in a context of immunodeficiency
hurwitz 2010-02-10 22:02	I think we all agree on the diagnosis of an atypical plasma cell hyperplasia in the context of an immune definciency, possibly therapy related.
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