Clinicopathologic features of plasmablastic lymphoma

Single-center series of 8 cases from Saudi Arabia

Ghaleb Elyamany, Ali Matar Alzahrani, Muna Aljuboury, Najlah mogadem, Nagham Rehan, Omar Alsuhaibani, Abdulaziz Alabdulaaly, Eman Al-Mussaed, Imad Elhag, Abdullah AlFiaar

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Abstract

Background: Plasmablastic lymphoma (PBL) is a rare subtype of non-Hodgkin's lymphoma. Characterized by its aggressive nature and plasmacytic differentiation, PBL remains a therapeutic and diagnostic challenge; it generally has a poor prognosis with very few long-term survivors and most patients dying within 2 years from initial presentation. PBL has been reported in several other countries; however, there have been no reportedcases from Saudi Arabia. Here, we report 8 cases of PBL depicting the clinical presentation, immunocompetency, immunphenotypic characterization, diagnostic challenges and treatment outcome. Methods: The medical records were reviewed for clinical presentation, staging, laboratory data, radiological studies, treatments, and outcomes. A broad immunohistochemical panel consisting of CD45, CD3, CD20, CD79a, Pax5, CD38, CD138, MUM1, EMA, Kappa, Lambda, CD 56, CD30, Bcl-2, Bcl-6, Alk-1, Ki-67, EBV-LMP-1, and HHV8 was performed. Results: The tumors predominantly exhibited immunoblastic/plasmablastic or plasmacytic morphologic features and had a plasma cell-like immunophenotype. All cases were immunoreactive for CD38, CD138 and MUM1 confirming plasma cell differentiation of the tumor cells. CD20 was negative for all cases; whereas CD79a and Pax5 were weakly positive in 2cases. All 8 cases were EBV-LMP-1/EBER-1 negative, and 1 case was HHV8 positive. Similar to previously published studies, PBL in Saudi Arabia is characterized by male predominance (6/8), median age 51.5 years (mean age 46 years), associated with early dissemination, poor response to therapy, and limited survival (average survival time, 6.4 months, median overall survival 5.5 months). However, it does have some unique features. It occurs more commonly in immunocompetent persons (6/8, 75 %), is not associated with EBV infection (0/8), and nodal involvement (either primary or secondary) is common among patients (6/8). In addition, extra-oral sites are more common than oral/nasal cavities (7/8) and the c-myc gene is not common (1/8, 12.5 %). Conclusion: It appears that PBL is heterogeneous in terms of clinical presentation and morphology. PBL is a therapeutic challenge with a clinical course that is characterized by its high rate of relapse and death. To date, treatment responses are usually partial and temporary. Therapies that are more intensive than CHOP do not seem to prolong survival. Further research is needed to understand the biology and molecular pathogenesis of PBL in order to improve therapies. Virtual slides: The virtual slides for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1465801416161912

Original languageEnglish
Article number78
JournalDiagnostic Pathology
Volume10
Issue number1
DOIs
Publication statusPublished - 25 Jun 2015

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Saudi Arabia
Survival
Human Herpesvirus 4
Therapeutics
myc Genes
Epstein-Barr Virus Infections
Plasmacytoma
Plasmablastic Lymphoma
Nasal Cavity
Plasma Cells
Non-Hodgkin's Lymphoma
Medical Records
Survivors
Mouth
Molecular Biology
Cell Differentiation
Recurrence
Mortality
Research

Keywords

  • Chemotherapy
  • HIV
  • Outcome
  • Plasmablastic lymphoma

Cite this

Elyamany, G., Alzahrani, A. M., Aljuboury, M., mogadem, N., Rehan, N., Alsuhaibani, O., ... AlFiaar, A. (2015). Clinicopathologic features of plasmablastic lymphoma: Single-center series of 8 cases from Saudi Arabia. Diagnostic Pathology, 10(1), [78]. https://doi.org/10.1186/s13000-015-0315-z
Elyamany, Ghaleb ; Alzahrani, Ali Matar ; Aljuboury, Muna ; mogadem, Najlah ; Rehan, Nagham ; Alsuhaibani, Omar ; Alabdulaaly, Abdulaziz ; Al-Mussaed, Eman ; Elhag, Imad ; AlFiaar, Abdullah. / Clinicopathologic features of plasmablastic lymphoma : Single-center series of 8 cases from Saudi Arabia. In: Diagnostic Pathology. 2015 ; Vol. 10, No. 1.
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title = "Clinicopathologic features of plasmablastic lymphoma: Single-center series of 8 cases from Saudi Arabia",
abstract = "Background: Plasmablastic lymphoma (PBL) is a rare subtype of non-Hodgkin's lymphoma. Characterized by its aggressive nature and plasmacytic differentiation, PBL remains a therapeutic and diagnostic challenge; it generally has a poor prognosis with very few long-term survivors and most patients dying within 2 years from initial presentation. PBL has been reported in several other countries; however, there have been no reportedcases from Saudi Arabia. Here, we report 8 cases of PBL depicting the clinical presentation, immunocompetency, immunphenotypic characterization, diagnostic challenges and treatment outcome. Methods: The medical records were reviewed for clinical presentation, staging, laboratory data, radiological studies, treatments, and outcomes. A broad immunohistochemical panel consisting of CD45, CD3, CD20, CD79a, Pax5, CD38, CD138, MUM1, EMA, Kappa, Lambda, CD 56, CD30, Bcl-2, Bcl-6, Alk-1, Ki-67, EBV-LMP-1, and HHV8 was performed. Results: The tumors predominantly exhibited immunoblastic/plasmablastic or plasmacytic morphologic features and had a plasma cell-like immunophenotype. All cases were immunoreactive for CD38, CD138 and MUM1 confirming plasma cell differentiation of the tumor cells. CD20 was negative for all cases; whereas CD79a and Pax5 were weakly positive in 2cases. All 8 cases were EBV-LMP-1/EBER-1 negative, and 1 case was HHV8 positive. Similar to previously published studies, PBL in Saudi Arabia is characterized by male predominance (6/8), median age 51.5 years (mean age 46 years), associated with early dissemination, poor response to therapy, and limited survival (average survival time, 6.4 months, median overall survival 5.5 months). However, it does have some unique features. It occurs more commonly in immunocompetent persons (6/8, 75 {\%}), is not associated with EBV infection (0/8), and nodal involvement (either primary or secondary) is common among patients (6/8). In addition, extra-oral sites are more common than oral/nasal cavities (7/8) and the c-myc gene is not common (1/8, 12.5 {\%}). Conclusion: It appears that PBL is heterogeneous in terms of clinical presentation and morphology. PBL is a therapeutic challenge with a clinical course that is characterized by its high rate of relapse and death. To date, treatment responses are usually partial and temporary. Therapies that are more intensive than CHOP do not seem to prolong survival. Further research is needed to understand the biology and molecular pathogenesis of PBL in order to improve therapies. Virtual slides: The virtual slides for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1465801416161912",
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author = "Ghaleb Elyamany and Alzahrani, {Ali Matar} and Muna Aljuboury and Najlah mogadem and Nagham Rehan and Omar Alsuhaibani and Abdulaziz Alabdulaaly and Eman Al-Mussaed and Imad Elhag and Abdullah AlFiaar",
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Elyamany, G, Alzahrani, AM, Aljuboury, M, mogadem, N, Rehan, N, Alsuhaibani, O, Alabdulaaly, A, Al-Mussaed, E, Elhag, I & AlFiaar, A 2015, 'Clinicopathologic features of plasmablastic lymphoma: Single-center series of 8 cases from Saudi Arabia', Diagnostic Pathology, vol. 10, no. 1, 78. https://doi.org/10.1186/s13000-015-0315-z

Clinicopathologic features of plasmablastic lymphoma : Single-center series of 8 cases from Saudi Arabia. / Elyamany, Ghaleb; Alzahrani, Ali Matar; Aljuboury, Muna; mogadem, Najlah; Rehan, Nagham; Alsuhaibani, Omar; Alabdulaaly, Abdulaziz; Al-Mussaed, Eman; Elhag, Imad; AlFiaar, Abdullah.

In: Diagnostic Pathology, Vol. 10, No. 1, 78, 25.06.2015.

Research output: Contribution to journalArticleResearchpeer-review

TY - JOUR

T1 - Clinicopathologic features of plasmablastic lymphoma

T2 - Single-center series of 8 cases from Saudi Arabia

AU - Elyamany, Ghaleb

AU - Alzahrani, Ali Matar

AU - Aljuboury, Muna

AU - mogadem, Najlah

AU - Rehan, Nagham

AU - Alsuhaibani, Omar

AU - Alabdulaaly, Abdulaziz

AU - Al-Mussaed, Eman

AU - Elhag, Imad

AU - AlFiaar, Abdullah

PY - 2015/6/25

Y1 - 2015/6/25

N2 - Background: Plasmablastic lymphoma (PBL) is a rare subtype of non-Hodgkin's lymphoma. Characterized by its aggressive nature and plasmacytic differentiation, PBL remains a therapeutic and diagnostic challenge; it generally has a poor prognosis with very few long-term survivors and most patients dying within 2 years from initial presentation. PBL has been reported in several other countries; however, there have been no reportedcases from Saudi Arabia. Here, we report 8 cases of PBL depicting the clinical presentation, immunocompetency, immunphenotypic characterization, diagnostic challenges and treatment outcome. Methods: The medical records were reviewed for clinical presentation, staging, laboratory data, radiological studies, treatments, and outcomes. A broad immunohistochemical panel consisting of CD45, CD3, CD20, CD79a, Pax5, CD38, CD138, MUM1, EMA, Kappa, Lambda, CD 56, CD30, Bcl-2, Bcl-6, Alk-1, Ki-67, EBV-LMP-1, and HHV8 was performed. Results: The tumors predominantly exhibited immunoblastic/plasmablastic or plasmacytic morphologic features and had a plasma cell-like immunophenotype. All cases were immunoreactive for CD38, CD138 and MUM1 confirming plasma cell differentiation of the tumor cells. CD20 was negative for all cases; whereas CD79a and Pax5 were weakly positive in 2cases. All 8 cases were EBV-LMP-1/EBER-1 negative, and 1 case was HHV8 positive. Similar to previously published studies, PBL in Saudi Arabia is characterized by male predominance (6/8), median age 51.5 years (mean age 46 years), associated with early dissemination, poor response to therapy, and limited survival (average survival time, 6.4 months, median overall survival 5.5 months). However, it does have some unique features. It occurs more commonly in immunocompetent persons (6/8, 75 %), is not associated with EBV infection (0/8), and nodal involvement (either primary or secondary) is common among patients (6/8). In addition, extra-oral sites are more common than oral/nasal cavities (7/8) and the c-myc gene is not common (1/8, 12.5 %). Conclusion: It appears that PBL is heterogeneous in terms of clinical presentation and morphology. PBL is a therapeutic challenge with a clinical course that is characterized by its high rate of relapse and death. To date, treatment responses are usually partial and temporary. Therapies that are more intensive than CHOP do not seem to prolong survival. Further research is needed to understand the biology and molecular pathogenesis of PBL in order to improve therapies. Virtual slides: The virtual slides for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1465801416161912

AB - Background: Plasmablastic lymphoma (PBL) is a rare subtype of non-Hodgkin's lymphoma. Characterized by its aggressive nature and plasmacytic differentiation, PBL remains a therapeutic and diagnostic challenge; it generally has a poor prognosis with very few long-term survivors and most patients dying within 2 years from initial presentation. PBL has been reported in several other countries; however, there have been no reportedcases from Saudi Arabia. Here, we report 8 cases of PBL depicting the clinical presentation, immunocompetency, immunphenotypic characterization, diagnostic challenges and treatment outcome. Methods: The medical records were reviewed for clinical presentation, staging, laboratory data, radiological studies, treatments, and outcomes. A broad immunohistochemical panel consisting of CD45, CD3, CD20, CD79a, Pax5, CD38, CD138, MUM1, EMA, Kappa, Lambda, CD 56, CD30, Bcl-2, Bcl-6, Alk-1, Ki-67, EBV-LMP-1, and HHV8 was performed. Results: The tumors predominantly exhibited immunoblastic/plasmablastic or plasmacytic morphologic features and had a plasma cell-like immunophenotype. All cases were immunoreactive for CD38, CD138 and MUM1 confirming plasma cell differentiation of the tumor cells. CD20 was negative for all cases; whereas CD79a and Pax5 were weakly positive in 2cases. All 8 cases were EBV-LMP-1/EBER-1 negative, and 1 case was HHV8 positive. Similar to previously published studies, PBL in Saudi Arabia is characterized by male predominance (6/8), median age 51.5 years (mean age 46 years), associated with early dissemination, poor response to therapy, and limited survival (average survival time, 6.4 months, median overall survival 5.5 months). However, it does have some unique features. It occurs more commonly in immunocompetent persons (6/8, 75 %), is not associated with EBV infection (0/8), and nodal involvement (either primary or secondary) is common among patients (6/8). In addition, extra-oral sites are more common than oral/nasal cavities (7/8) and the c-myc gene is not common (1/8, 12.5 %). Conclusion: It appears that PBL is heterogeneous in terms of clinical presentation and morphology. PBL is a therapeutic challenge with a clinical course that is characterized by its high rate of relapse and death. To date, treatment responses are usually partial and temporary. Therapies that are more intensive than CHOP do not seem to prolong survival. Further research is needed to understand the biology and molecular pathogenesis of PBL in order to improve therapies. Virtual slides: The virtual slides for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1465801416161912

KW - Chemotherapy

KW - HIV

KW - Outcome

KW - Plasmablastic lymphoma

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VL - 10

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