KSHV- and EBV-associated germinotropic lymphoproliferative disorder

Ming Qing Du, Tim C. Diss, Hongxiang Liu, Hongtao Ye, Rifat A. Hamoudi, José Cabeçadas, Henry Y. Dong, Nancy Lee Harris, John K C Chan, John W. Rees, Ahmet Dogan, Peter G. Isaacson

Research output: Contribution to journalArticle

153 Citations (Scopus)

Abstract

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."

Original languageEnglish (US)
Pages (from-to)3415-3418
Number of pages4
JournalBlood
Volume100
Issue number9
DOIs
StatePublished - Nov 1 2002
Externally publishedYes

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Human Herpesvirus 8
Lymphoproliferative Disorders
Human Herpesvirus 4
Immunoglobulins
Genes
Immunoglobulin Genes
Germinal Center
Immunoglobulin Heavy Chains
Chemotherapy
Radiotherapy
Immunoglobulin A
Primary Effusion Lymphoma
Interleukin-6
Oligoclonal Bands
Cells
Tissue
Gene Rearrangement
B-Lymphocytes
Light
Drug Therapy

ASJC Scopus subject areas

  • Hematology

Cite this

Du, M. Q., Diss, T. C., Liu, H., Ye, H., Hamoudi, R. A., Cabeçadas, J., ... Isaacson, P. G. (2002). KSHV- and EBV-associated germinotropic lymphoproliferative disorder. Blood, 100(9), 3415-3418. https://doi.org/10.1182/blood-2002-02-0487

KSHV- and EBV-associated germinotropic lymphoproliferative disorder. / 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.

In: Blood, Vol. 100, No. 9, 01.11.2002, p. 3415-3418.

Research output: Contribution to journalArticle

Du, MQ, Diss, TC, Liu, H, Ye, H, Hamoudi, RA, Cabeçadas, J, Dong, HY, Harris, NL, Chan, JKC, Rees, JW, Dogan, A & Isaacson, PG 2002, 'KSHV- and EBV-associated germinotropic lymphoproliferative disorder', Blood, vol. 100, no. 9, pp. 3415-3418. https://doi.org/10.1182/blood-2002-02-0487
Du MQ, Diss TC, Liu H, Ye H, Hamoudi RA, Cabeçadas J et al. KSHV- and EBV-associated germinotropic lymphoproliferative disorder. Blood. 2002 Nov 1;100(9):3415-3418. https://doi.org/10.1182/blood-2002-02-0487
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. / KSHV- and EBV-associated germinotropic lymphoproliferative disorder. In: Blood. 2002 ; Vol. 100, No. 9. pp. 3415-3418.
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AU - Du, Ming Qing

AU - Diss, Tim C.

AU - Liu, Hongxiang

AU - Ye, Hongtao

AU - Hamoudi, Rifat A.

AU - Cabeçadas, José

AU - Dong, Henry Y.

AU - Harris, Nancy Lee

AU - Chan, John K C

AU - Rees, John W.

AU - Dogan, Ahmet

AU - Isaacson, Peter G.

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N2 - 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."

AB - 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."

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