A CLINICO-PATHOLOGIC AND IMMUNOHISTOCHEMICAL ANALYSIS OF ORAL AND EXTRA-ORAL PLASMABLASTIC LYMPHOMA IN SOUTH AFRICA
(Abstract release date: 05/19/16)
EHA Library. Meer S. 06/09/16; 132515; E966
Prof. Shabnum Meer
Contributions
Contributions
Abstract
Abstract: E966
Type: Eposter Presentation
Background
Plasmablastic lymphomas (PBLs) are a group of aggressive non-Hodgkin lymphomas originally described to be exclusive to the oral cavity in patients with immunodeficiency. This group of uncommon lymphomas, which shows morphologic, phenotypic and molecular features of terminally differentiated B-cells is increasingly being described in many extra-oral sites as well, in both HIV and non-HIV infected patients. Data relating to age, gender, HIV status and immunophenotypic profile have been published in case reports, case series and systemic reviews. To date there are no data on extra-oral PBLs in South Africa where HIV is higly endemic. We present a series of 101 oral (55) and extra-oral (45) PBLs which forms the largest global series described to date in a region of Africa where HIV is endemic.
Aims
To characterize the clinical, morphological and immunological features of oral and extra-oral plasmablastic lymphomas in a defined South African population.
Methods
This is a retrospective study on archival cases of extra-oral and oral PBLs diagnosed over the period 2006 to 2015 in the Department of Anatomical Pathology, University of the Witwatersrand, Johannesburg, South Africa. Clinical parameters analyzed included patients age, gender, HIV status and site of tumor presentation. Each case was reviewed histologically to confirm the diagnosis and assessed immunohistochemically with CD45 (LCA), CD20, CD79a, PAX5, CD138, MUM1, BLIMP1, VS38c, Ki-67, BCL6, CD10, and HHV8 using standard immunohistochemistry protocols. The presence of EBV-encoded early nuclear RNAs (EBER1 and EBER2) was assessed by chromogenic in-situ hybridization. Ethical clearance was obtained from the University of the Witwatersrand human research ethics committee.
Results
Forty-six patients were diagnosed with extra-oral PBLs and 55 patients with oral PBLs. These included all adult patients, except for one case of a nine-year old child. The age ranged from 9 to 59 years (mean:35.5; median:35) for extra-oral cases and 22 to 78 years for oral PBLs (mean:41.1; median:41). Of the 18 and 34 patients with extra-oral and oral PBLS with known HIV status, 17 (95%) and 33 (97%) patients respectively were infected with HIV. None of the patients had systemic disease at presentation. The overall male:female ratio was 1.9:1 (67 males and 34 females), with 66% of PBLs occurring in males. The male:female ratio for extra-oral and oral PBLs was 1.4:1 (27 males (59%) and 19 females) and 2.7:1 (40 (73%) males and 15 females) respectively. The anus was the favoured extra-oral site of presentation (13 of 46 cases, 28%), followed by soft tissue (11 of 46 cases, 24%). The favoured oral site for PBL was the maxilla (16 of 55 cases, 29%) followed by the palate (11 of 55 cases; 20%). The histomorphology in both oral and extra-oral PBLs was similar showing both plasmablastic and plasmacytic features. The immunohistochemical profile of all PBLs in the study recapitulated that found for both oral and extra-oral PBL in the literature, except for CD45 (leucocyte common antigen), which signalled positively in a higher percentage of cases. Overall 73 of 80 cases (91%) were positive for CD45; 36 of 42 extra-oral cases (85.7%) and 37 of 38 oral cases (97%). The positive membrane signal for CD45 was of variable intensity, between 5 and 100% of tumor cells. EBV was positive by in-situ hybridization in 71 of 74 cases (96%), i.e. in 37 of 40 (93%) extra-oral cases and all 34 oral PBLs (100%).
Conclusion
Extra-oral PBL was identical to its oral counterpart in gender and age distribution, HIV status, morphological appearances, immunophenotypic profile and association with EBV. The high association with EBV as assessed by in-situ hybridization studies mirrors that of extra-oral and oral PBLs reported in the literature. A peculiarity observed within this case cohort was the high level of expression of CD45. This has been reported to be of low or near absent in most cases of PBL, as defined by the WHO 2008 and recorded in the literature. Although the HIV status was only known for approximately half of these cases, HIV associated PBL manifested as a single disease in both oral and extra-oral forms, and should be regarded as the same tumor.
Session topic: E-poster
Keyword(s): HIV, Non-Hodgkin's lymphoma
Type: Eposter Presentation
Background
Plasmablastic lymphomas (PBLs) are a group of aggressive non-Hodgkin lymphomas originally described to be exclusive to the oral cavity in patients with immunodeficiency. This group of uncommon lymphomas, which shows morphologic, phenotypic and molecular features of terminally differentiated B-cells is increasingly being described in many extra-oral sites as well, in both HIV and non-HIV infected patients. Data relating to age, gender, HIV status and immunophenotypic profile have been published in case reports, case series and systemic reviews. To date there are no data on extra-oral PBLs in South Africa where HIV is higly endemic. We present a series of 101 oral (55) and extra-oral (45) PBLs which forms the largest global series described to date in a region of Africa where HIV is endemic.
Aims
To characterize the clinical, morphological and immunological features of oral and extra-oral plasmablastic lymphomas in a defined South African population.
Methods
This is a retrospective study on archival cases of extra-oral and oral PBLs diagnosed over the period 2006 to 2015 in the Department of Anatomical Pathology, University of the Witwatersrand, Johannesburg, South Africa. Clinical parameters analyzed included patients age, gender, HIV status and site of tumor presentation. Each case was reviewed histologically to confirm the diagnosis and assessed immunohistochemically with CD45 (LCA), CD20, CD79a, PAX5, CD138, MUM1, BLIMP1, VS38c, Ki-67, BCL6, CD10, and HHV8 using standard immunohistochemistry protocols. The presence of EBV-encoded early nuclear RNAs (EBER1 and EBER2) was assessed by chromogenic in-situ hybridization. Ethical clearance was obtained from the University of the Witwatersrand human research ethics committee.
Results
Forty-six patients were diagnosed with extra-oral PBLs and 55 patients with oral PBLs. These included all adult patients, except for one case of a nine-year old child. The age ranged from 9 to 59 years (mean:35.5; median:35) for extra-oral cases and 22 to 78 years for oral PBLs (mean:41.1; median:41). Of the 18 and 34 patients with extra-oral and oral PBLS with known HIV status, 17 (95%) and 33 (97%) patients respectively were infected with HIV. None of the patients had systemic disease at presentation. The overall male:female ratio was 1.9:1 (67 males and 34 females), with 66% of PBLs occurring in males. The male:female ratio for extra-oral and oral PBLs was 1.4:1 (27 males (59%) and 19 females) and 2.7:1 (40 (73%) males and 15 females) respectively. The anus was the favoured extra-oral site of presentation (13 of 46 cases, 28%), followed by soft tissue (11 of 46 cases, 24%). The favoured oral site for PBL was the maxilla (16 of 55 cases, 29%) followed by the palate (11 of 55 cases; 20%). The histomorphology in both oral and extra-oral PBLs was similar showing both plasmablastic and plasmacytic features. The immunohistochemical profile of all PBLs in the study recapitulated that found for both oral and extra-oral PBL in the literature, except for CD45 (leucocyte common antigen), which signalled positively in a higher percentage of cases. Overall 73 of 80 cases (91%) were positive for CD45; 36 of 42 extra-oral cases (85.7%) and 37 of 38 oral cases (97%). The positive membrane signal for CD45 was of variable intensity, between 5 and 100% of tumor cells. EBV was positive by in-situ hybridization in 71 of 74 cases (96%), i.e. in 37 of 40 (93%) extra-oral cases and all 34 oral PBLs (100%).
Conclusion
Extra-oral PBL was identical to its oral counterpart in gender and age distribution, HIV status, morphological appearances, immunophenotypic profile and association with EBV. The high association with EBV as assessed by in-situ hybridization studies mirrors that of extra-oral and oral PBLs reported in the literature. A peculiarity observed within this case cohort was the high level of expression of CD45. This has been reported to be of low or near absent in most cases of PBL, as defined by the WHO 2008 and recorded in the literature. Although the HIV status was only known for approximately half of these cases, HIV associated PBL manifested as a single disease in both oral and extra-oral forms, and should be regarded as the same tumor.
Session topic: E-poster
Keyword(s): HIV, Non-Hodgkin's lymphoma
Abstract: E966
Type: Eposter Presentation
Background
Plasmablastic lymphomas (PBLs) are a group of aggressive non-Hodgkin lymphomas originally described to be exclusive to the oral cavity in patients with immunodeficiency. This group of uncommon lymphomas, which shows morphologic, phenotypic and molecular features of terminally differentiated B-cells is increasingly being described in many extra-oral sites as well, in both HIV and non-HIV infected patients. Data relating to age, gender, HIV status and immunophenotypic profile have been published in case reports, case series and systemic reviews. To date there are no data on extra-oral PBLs in South Africa where HIV is higly endemic. We present a series of 101 oral (55) and extra-oral (45) PBLs which forms the largest global series described to date in a region of Africa where HIV is endemic.
Aims
To characterize the clinical, morphological and immunological features of oral and extra-oral plasmablastic lymphomas in a defined South African population.
Methods
This is a retrospective study on archival cases of extra-oral and oral PBLs diagnosed over the period 2006 to 2015 in the Department of Anatomical Pathology, University of the Witwatersrand, Johannesburg, South Africa. Clinical parameters analyzed included patients age, gender, HIV status and site of tumor presentation. Each case was reviewed histologically to confirm the diagnosis and assessed immunohistochemically with CD45 (LCA), CD20, CD79a, PAX5, CD138, MUM1, BLIMP1, VS38c, Ki-67, BCL6, CD10, and HHV8 using standard immunohistochemistry protocols. The presence of EBV-encoded early nuclear RNAs (EBER1 and EBER2) was assessed by chromogenic in-situ hybridization. Ethical clearance was obtained from the University of the Witwatersrand human research ethics committee.
Results
Forty-six patients were diagnosed with extra-oral PBLs and 55 patients with oral PBLs. These included all adult patients, except for one case of a nine-year old child. The age ranged from 9 to 59 years (mean:35.5; median:35) for extra-oral cases and 22 to 78 years for oral PBLs (mean:41.1; median:41). Of the 18 and 34 patients with extra-oral and oral PBLS with known HIV status, 17 (95%) and 33 (97%) patients respectively were infected with HIV. None of the patients had systemic disease at presentation. The overall male:female ratio was 1.9:1 (67 males and 34 females), with 66% of PBLs occurring in males. The male:female ratio for extra-oral and oral PBLs was 1.4:1 (27 males (59%) and 19 females) and 2.7:1 (40 (73%) males and 15 females) respectively. The anus was the favoured extra-oral site of presentation (13 of 46 cases, 28%), followed by soft tissue (11 of 46 cases, 24%). The favoured oral site for PBL was the maxilla (16 of 55 cases, 29%) followed by the palate (11 of 55 cases; 20%). The histomorphology in both oral and extra-oral PBLs was similar showing both plasmablastic and plasmacytic features. The immunohistochemical profile of all PBLs in the study recapitulated that found for both oral and extra-oral PBL in the literature, except for CD45 (leucocyte common antigen), which signalled positively in a higher percentage of cases. Overall 73 of 80 cases (91%) were positive for CD45; 36 of 42 extra-oral cases (85.7%) and 37 of 38 oral cases (97%). The positive membrane signal for CD45 was of variable intensity, between 5 and 100% of tumor cells. EBV was positive by in-situ hybridization in 71 of 74 cases (96%), i.e. in 37 of 40 (93%) extra-oral cases and all 34 oral PBLs (100%).
Conclusion
Extra-oral PBL was identical to its oral counterpart in gender and age distribution, HIV status, morphological appearances, immunophenotypic profile and association with EBV. The high association with EBV as assessed by in-situ hybridization studies mirrors that of extra-oral and oral PBLs reported in the literature. A peculiarity observed within this case cohort was the high level of expression of CD45. This has been reported to be of low or near absent in most cases of PBL, as defined by the WHO 2008 and recorded in the literature. Although the HIV status was only known for approximately half of these cases, HIV associated PBL manifested as a single disease in both oral and extra-oral forms, and should be regarded as the same tumor.
Session topic: E-poster
Keyword(s): HIV, Non-Hodgkin's lymphoma
Type: Eposter Presentation
Background
Plasmablastic lymphomas (PBLs) are a group of aggressive non-Hodgkin lymphomas originally described to be exclusive to the oral cavity in patients with immunodeficiency. This group of uncommon lymphomas, which shows morphologic, phenotypic and molecular features of terminally differentiated B-cells is increasingly being described in many extra-oral sites as well, in both HIV and non-HIV infected patients. Data relating to age, gender, HIV status and immunophenotypic profile have been published in case reports, case series and systemic reviews. To date there are no data on extra-oral PBLs in South Africa where HIV is higly endemic. We present a series of 101 oral (55) and extra-oral (45) PBLs which forms the largest global series described to date in a region of Africa where HIV is endemic.
Aims
To characterize the clinical, morphological and immunological features of oral and extra-oral plasmablastic lymphomas in a defined South African population.
Methods
This is a retrospective study on archival cases of extra-oral and oral PBLs diagnosed over the period 2006 to 2015 in the Department of Anatomical Pathology, University of the Witwatersrand, Johannesburg, South Africa. Clinical parameters analyzed included patients age, gender, HIV status and site of tumor presentation. Each case was reviewed histologically to confirm the diagnosis and assessed immunohistochemically with CD45 (LCA), CD20, CD79a, PAX5, CD138, MUM1, BLIMP1, VS38c, Ki-67, BCL6, CD10, and HHV8 using standard immunohistochemistry protocols. The presence of EBV-encoded early nuclear RNAs (EBER1 and EBER2) was assessed by chromogenic in-situ hybridization. Ethical clearance was obtained from the University of the Witwatersrand human research ethics committee.
Results
Forty-six patients were diagnosed with extra-oral PBLs and 55 patients with oral PBLs. These included all adult patients, except for one case of a nine-year old child. The age ranged from 9 to 59 years (mean:35.5; median:35) for extra-oral cases and 22 to 78 years for oral PBLs (mean:41.1; median:41). Of the 18 and 34 patients with extra-oral and oral PBLS with known HIV status, 17 (95%) and 33 (97%) patients respectively were infected with HIV. None of the patients had systemic disease at presentation. The overall male:female ratio was 1.9:1 (67 males and 34 females), with 66% of PBLs occurring in males. The male:female ratio for extra-oral and oral PBLs was 1.4:1 (27 males (59%) and 19 females) and 2.7:1 (40 (73%) males and 15 females) respectively. The anus was the favoured extra-oral site of presentation (13 of 46 cases, 28%), followed by soft tissue (11 of 46 cases, 24%). The favoured oral site for PBL was the maxilla (16 of 55 cases, 29%) followed by the palate (11 of 55 cases; 20%). The histomorphology in both oral and extra-oral PBLs was similar showing both plasmablastic and plasmacytic features. The immunohistochemical profile of all PBLs in the study recapitulated that found for both oral and extra-oral PBL in the literature, except for CD45 (leucocyte common antigen), which signalled positively in a higher percentage of cases. Overall 73 of 80 cases (91%) were positive for CD45; 36 of 42 extra-oral cases (85.7%) and 37 of 38 oral cases (97%). The positive membrane signal for CD45 was of variable intensity, between 5 and 100% of tumor cells. EBV was positive by in-situ hybridization in 71 of 74 cases (96%), i.e. in 37 of 40 (93%) extra-oral cases and all 34 oral PBLs (100%).
Conclusion
Extra-oral PBL was identical to its oral counterpart in gender and age distribution, HIV status, morphological appearances, immunophenotypic profile and association with EBV. The high association with EBV as assessed by in-situ hybridization studies mirrors that of extra-oral and oral PBLs reported in the literature. A peculiarity observed within this case cohort was the high level of expression of CD45. This has been reported to be of low or near absent in most cases of PBL, as defined by the WHO 2008 and recorded in the literature. Although the HIV status was only known for approximately half of these cases, HIV associated PBL manifested as a single disease in both oral and extra-oral forms, and should be regarded as the same tumor.
Session topic: E-poster
Keyword(s): HIV, Non-Hodgkin's lymphoma
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