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Atypical plasma cell proliferation, possibly in a therapy induced immunedefficiency, TLGL? M /37 (12275) » Granuloma CD68.jpg
Filename: Granuloma CD68.jpg
[Atypical plasma cell proliferation, possibly in a therapy induced immunedefficiency, TLGL? M /37]
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.
Sender: ugnius
2009-12-22 10:55
INCTR - EBMWG Hematopathology Online

Last modified: 2009-12-22 11:45:05