HHV8/EBV Coinfection Lymphoproliferative Disorder: Rare Entity with a Favorable Outcome
HHV8/EBV-associated germinotropic lymphoproliferative disorder (GLD) is a challenging diagnosis given its rarity, the particular clinical presentation, and the lack of expression of markers usually used in establishing hematopoietic lineage. We report a new case of HHV8/EBV GLD in an immunocompetent...
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| Format: | Article |
| Language: | English |
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Wiley
2017-01-01
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| Series: | Case Reports in Hematology |
| Online Access: | http://dx.doi.org/10.1155/2017/1578429 |
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| author | Dhouha Bacha Beya Chelly Houda Kilani Lamia Charfi Amel Douggaz Samia Chatti Emna Chelbi |
| author_facet | Dhouha Bacha Beya Chelly Houda Kilani Lamia Charfi Amel Douggaz Samia Chatti Emna Chelbi |
| author_sort | Dhouha Bacha |
| collection | DOAJ |
| description | HHV8/EBV-associated germinotropic lymphoproliferative disorder (GLD) is a challenging diagnosis given its rarity, the particular clinical presentation, and the lack of expression of markers usually used in establishing hematopoietic lineage. We report a new case of HHV8/EBV GLD in an immunocompetent 78-year-old woman. The diagnosis was made in an incidentally discovered lymphadenopathy. Histological examination showed a nodular lymphoid proliferation centered by aggregates of atypical plasmablastic cells admixed with small lymphoid cells. Tumor cells were strongly positive with EMA, HHV8, LMP1, CD38, CD138, and kappa light chains. They were negative with common lymphoma-associated markers (CD20, CD3, CD15, CD30, CD10, and bcl2). In situ hybridization confirmed the monotypic kappa light chains and the EBV infection (EBER+). A polyclonal pattern of Ig gene rearrangement was detected by PCR analysis. In the adjacent lymph node parenchyma, some germinal centers mimicked Castleman disease. In this case, the differential diagnosis was discussed with an early stage of large B-cell lymphoma arising in HHV8-associated multicentric Castleman disease. The clinical presentation, the immunophenotype, and the molecular results helped to make the accurate diagnosis. Through the review of the nine previously reported cases in literature, we discuss the clinical and pathologic features and the differential diagnosis of HHV8/EBV GLD. |
| format | Article |
| id | doaj-art-38374fea4b4b43e2a9608513031a50fe |
| institution | Kabale University |
| issn | 2090-6560 2090-6579 |
| language | English |
| publishDate | 2017-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Hematology |
| spelling | doaj-art-38374fea4b4b43e2a9608513031a50fe2025-02-03T07:24:35ZengWileyCase Reports in Hematology2090-65602090-65792017-01-01201710.1155/2017/15784291578429HHV8/EBV Coinfection Lymphoproliferative Disorder: Rare Entity with a Favorable OutcomeDhouha Bacha0Beya Chelly1Houda Kilani2Lamia Charfi3Amel Douggaz4Samia Chatti5Emna Chelbi6Pathology Department, Mohamed Tahar Maamouri Hospital, Mrezga, Nabeul, TunisiaPathology Department, Mohamed Tahar Maamouri Hospital, Mrezga, Nabeul, TunisiaPathology Department, Mohamed Tahar Maamouri Hospital, Mrezga, Nabeul, TunisiaPathology Department, Mohamed Tahar Maamouri Hospital, Mrezga, Nabeul, TunisiaPathology Department, Mohamed Tahar Maamouri Hospital, Mrezga, Nabeul, TunisiaPathology Department, Mohamed Tahar Maamouri Hospital, Mrezga, Nabeul, TunisiaPathology Department, Mohamed Tahar Maamouri Hospital, Mrezga, Nabeul, TunisiaHHV8/EBV-associated germinotropic lymphoproliferative disorder (GLD) is a challenging diagnosis given its rarity, the particular clinical presentation, and the lack of expression of markers usually used in establishing hematopoietic lineage. We report a new case of HHV8/EBV GLD in an immunocompetent 78-year-old woman. The diagnosis was made in an incidentally discovered lymphadenopathy. Histological examination showed a nodular lymphoid proliferation centered by aggregates of atypical plasmablastic cells admixed with small lymphoid cells. Tumor cells were strongly positive with EMA, HHV8, LMP1, CD38, CD138, and kappa light chains. They were negative with common lymphoma-associated markers (CD20, CD3, CD15, CD30, CD10, and bcl2). In situ hybridization confirmed the monotypic kappa light chains and the EBV infection (EBER+). A polyclonal pattern of Ig gene rearrangement was detected by PCR analysis. In the adjacent lymph node parenchyma, some germinal centers mimicked Castleman disease. In this case, the differential diagnosis was discussed with an early stage of large B-cell lymphoma arising in HHV8-associated multicentric Castleman disease. The clinical presentation, the immunophenotype, and the molecular results helped to make the accurate diagnosis. Through the review of the nine previously reported cases in literature, we discuss the clinical and pathologic features and the differential diagnosis of HHV8/EBV GLD.http://dx.doi.org/10.1155/2017/1578429 |
| spellingShingle | Dhouha Bacha Beya Chelly Houda Kilani Lamia Charfi Amel Douggaz Samia Chatti Emna Chelbi HHV8/EBV Coinfection Lymphoproliferative Disorder: Rare Entity with a Favorable Outcome Case Reports in Hematology |
| title | HHV8/EBV Coinfection Lymphoproliferative Disorder: Rare Entity with a Favorable Outcome |
| title_full | HHV8/EBV Coinfection Lymphoproliferative Disorder: Rare Entity with a Favorable Outcome |
| title_fullStr | HHV8/EBV Coinfection Lymphoproliferative Disorder: Rare Entity with a Favorable Outcome |
| title_full_unstemmed | HHV8/EBV Coinfection Lymphoproliferative Disorder: Rare Entity with a Favorable Outcome |
| title_short | HHV8/EBV Coinfection Lymphoproliferative Disorder: Rare Entity with a Favorable Outcome |
| title_sort | hhv8 ebv coinfection lymphoproliferative disorder rare entity with a favorable outcome |
| url | http://dx.doi.org/10.1155/2017/1578429 |
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