RETROSPECTIVE ANALYSIS:  
28yrs lady, presenting with ulcerations/destruction of oral mucosa, palate. The multiple biopsies (2001, 2002) from oral mucosa, hypopharynx, palate were taken with clinical diagnoses "Wegener granulomatosis" and "lethal midline granuloma". HISTO: on the slides (2 biopsy series). DIAGNOSIS of lymphoma WAS NOT DONE (2001, 2002).  
AUTOPSY (2002) data: The destruction and exulceration of palate durum and molle, tissues of left mandibular processus alveolaris and gingival mucosae with confluent necrosis and diffuse perivascular infiltration of atypical small- medium-large lymphocytes with irregular nuclei were present with the admixture of granulocytes, plasmacytes. Diagnosis was established (below).  
LIVER HISTOLOGY: The lymphoid infiltration in liver consisting from atypical small- medium-large lymphocytes with irregular nuclei were present. The cells were larger and pleorphism higher that in previous biopsy material.  
LUNG HISTOLOGY: The scant intraalveolar haemorhages and scant perivascular neoplastic infiltrates in the interstitium were present.  
IH (infiltrate)(RETROSPECTIVE): CD3 (cytoplasmic ε chain)/CD2/CD45RO, GranzymB/CD56 (+) C20/CD4/CD8/CD5/CD7/CD30/CD57 (-). Ki67 about 30%. EBV LMP1 (+) within single large cell CD30 cytoplasmic reaction only in liver. Immunoreactivity was significantly lower in autopsy tissues. CD16, TCR β, TCR δ immunohistochemical stains and clonality analysis were not applied (unavailable).  
PROPOSED DIAGNOSIS: NK/T extranodal lymphoma, nasal type.  
DISCUSSION:1. Differential with other CD56+ cytotox.m.+ T NHL. 2. Liver and lung infiltrates shows more pleomorphic scattered (some- EBV LMP1 and CD30+) cells, than in biopsies (EBV LMP1 (-)). DIAGNOSTIC clues in small primary biopsy.

Mueller-Hermelink 2006-09-06 23:49

This is a very good teaching case for nasal NK/T cell lymphoma ( There might be some discussion whether you include hard palate and hypopharynx into nasal or attribute it to nasal-type). The morphology is brillant , even in the autopsy. It is of interest that liver involvement is portal ( and not sinusoidal like in hepatosplenic lymphoma or in large granular leukemia ). If one thinks of the entity the proof is easy , even in small biopsies : CD56 + , cytoplasmic CD3 , highly proliferative , angiocentric , cytotoxic proliferation , consistently EBER positive. Sometimes there are T cell phenotypes ( about 20% of cases) that can be CD56 negative , but still EBER positive showing TCR rearrangement. The nasal biosies usually have a high inflammatory background and one has to concentrate on the perivascular infiltrate.LMP is problematic : you may find some + cells , but it may also be completely negative due to latency type I of EBV.

ugnius 2006-09-07 09:22

Thank you Prof.Muller-Hermelink. In our country CD56, CD2 and EBV LMP1 was introduced in 2003; GranzymB, in 2004, CD57 in 2005 year. So conventional and IH experience dealing with certain lymphoproliferations was not so high. I know only 2 cases with this T/NK proliferation for 11 years from now so this item remains so rear...