[1333] Extracavitary KSHV+ Large B-Cell Lymphoma: Time To Have a New Name?

Zenggang Pan, Zhengbin Lu, Lanting Liu, David B Wilson, Vishnu Reddy, Huanyou Wang, Yongsheng Ren. Univ. of Alabama at Birmingham; Ameripath, Indianapolis, IN; Univ. of California San Diego

Background: Extracavitary Kaposi sarcoma-associated herpesvirus (KSHV)+ large B-cell lymphoma (EK-LBL) has rarely been observed without effusions. It is uncertain whether it should be considered as a separated entity or a variant of primary effusion lymphoma (PEL). The diagnosis of EK-LBL can be challenging due to lack of a clear definition and a consensus diagnostic term. Here, we reviewed 46 such cases with emphasis on the clinicopathologic features.
Design: 46 EK-LBL cases were collected from the English literature (42) or our files (4). All cases had no effusions before or after presentation. Special studies of our cases included immunostains [CD45, CD20, CD79a, PAX5, CD3, MUM1, EMA, CD30, CD138, immunoglobulin (Ig) light chains, and KSHV], EBV(EBER), and T-cell receptor and Ig gene rearrangements. The results were compared with those summarized from 95 PELs in the literature.

Clinicopathologic features of EK-LBL and PEL
 EK-LBLClassical PEL
Gender (male/female)45:1 86:1 
HIV44/4696%87/9592%
KSHV46/46100%61/61100%
EBER32/4276%26/4065%
CD45 (LCA)29/3876%65/6994%
CD209/4620%4/755%
CD79a8/3126%0/130%
Ig kappa4/3013%7/3321%
Ig lambda10/3232%7/3321%
CD13822/3170%20/3361%
MUM110/10100%12/12100%
CD3020/3459%39/5670%
EMA13/2259%22/2976%
IgH rearrangement15/1788%30/4271%



Results: EK-LBL almost exclusively occurred in HIV+ male, mostly located in the lymph node (28/46), followed by gastrointestinal tract (17). Tumor cells typically exhibited immunoblastic, plasmablastic or anaplastic morphology, with CD45+ but lack of B-cell (CD20, CD79a and PAX5) or T-cell markers. Most cases were EBV+, CD30+, CD138+ and EMA+. It was clinically aggressive; 8 of 16 patients died in 45 days although a few had favorable response.
Classical PEL had similar morphology and immunophenotype to EK-LBL, but it rarely had associated mass lesions and was more often positive for CD45, CD30 and EMA.


Conclusions: Although EK-LBL is generally similar to PEL, it probably should be considered as a distinct entity with a diagnostic term of “extracavitary KSHV+ LBL”.
Category: Hematopathology

Wednesday, March 2, 2011 9:30 AM

Poster Session V # 177, Wednesday Morning

 

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