NDLI: KSHV- and EBV-associated germinotropic lymphoproliferative disorder

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KSHV- and EBV-associated germinotropic lymphoproliferative disorder

Content Provider	Scilit
Author	Du, Ming-Qing Diss, Tim C. Liu, Hongxiang Ye, Hongtao Hamoudi, Rifat A. Cabeçadas, José Dong, Henry Y. Harris, Nancy Lee Chan, John K. C. Rees, John W. Dogan, Ahmet Isaacson, Peter G.
Copyright Year	2002
Description	Kaposi sarcoma–associated herpesvirus (KSHV) is known to be associated with 3 distinct lymphoproliferative disorders: primary effusion lymphoma (PEL), multicentric Castleman disease (MCD), and MCD-associated plasmablastic lymphoma. We report 3 cases of a previously undescribed KSHV-associated lymphoproliferative disorder. The disease presented as localized lymphadenopathy and showed a favorable response to chemotherapy or radiotherapy. Histologically, the lymphoproliferation is characterized by plasmablasts that preferentially involved germinal centers of the lymphoid follicles, forming confluent aggregates. They were negative for CD20, CD27, CD79a, CD138, BCL6, and CD10 but showed monotypic κ or λ light chain. Clusters of CD10+CD20+ residual follicle center cells were identified in some of the follicles. The plasmablasts were positive for both KSHV and EBV, and most of them also expressed viral interleukin-6 (vIL-6). Unexpectedly, molecular analysis of whole tissue sections or microdissected KSHV-positive aggregates demonstrated a polyclonal or oligoclonal pattern of immunoglobulin (Ig) gene rearrangement. The plasmablasts showed somatic mutation and intraclonal variation in the rearranged Ig genes, and one case expressed switched Ig heavy chain (IgA), suggesting that they originated from germinal center B cells. We propose calling this distinctive entity “KSHV-associated germinotropic lymphoproliferative disorder.”
Related Links	http://www.bloodjournal.org/content/100/9/3415.full.pdf
Ending Page	3418
Page Count	4
Starting Page	3415
DOI	10.1182/blood-2002-02-0487
Journal	Blood
Issue Number	9
Volume Number	100
Language	English
Publisher	American Society of Hematology
Publisher Date	2002-11-01
Access Restriction	Open
Subject Keyword	Pathology Lymphoma Mcd Cd10 Cd20 Follicles Lymphoproliferative Disorder Kshv Rearranged Journal: Blood (Vol- 109, Issue- 9)
Content Type	Text
Resource Type	Article

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