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 reported cases 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%).
  
  
  CONCLUSIONS
  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.
  The virtual slides for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1465801416161912.
authors.affiliation: Department of Pathology and Laboratory Medicine, Prince Sultan Military Medical City, Riyadh, Saudi Arabia. ghalebelyamany@yahoo.com.|Department of Hematology and Blood Bank, Theodor Bilharz Research Institute, Giza, Egypt. ghalebelyamany@yahoo.com.
authors.forename: Ghaleb
authors.initials: G
authors.lastname: Elyamany
authors.affiliation: Department of Oncology, Prince Sultan Military Medical City, Riyadh, Saudi Arabia.
authors.forename: Ali Matar
authors.initials: AM
authors.lastname: Alzahrani
authors.affiliation: Department of Pathology and Laboratory Medicine, Prince Sultan Military Medical City, Riyadh, Saudi Arabia.
authors.forename: Muna
authors.initials: M
authors.lastname: Aljuboury
authors.affiliation: Department of Pathology and Laboratory Medicine, Prince Sultan Military Medical City, Riyadh, Saudi Arabia.
authors.forename: Najlah
authors.initials: N
authors.lastname: Mogadem
authors.affiliation: Department of Hematology and Blood Bank, Theodor Bilharz Research Institute, Giza, Egypt.
authors.forename: Nagham
authors.initials: N
authors.lastname: Rehan
authors.affiliation: Department of Pathology and Laboratory Medicine, Prince Sultan Military Medical City, Riyadh, Saudi Arabia.
authors.forename: Omar
authors.initials: O
authors.lastname: Alsuhaibani
authors.affiliation: Department of Pathology and Laboratory Medicine, Prince Sultan Military Medical City, Riyadh, Saudi Arabia.
authors.forename: Abdulaziz
authors.initials: A
authors.lastname: Alabdulaaly
authors.affiliation: Department of Basic Science, Princess Nourah Bint Abdulrahman, University, College of Medicine, Riyadh, Saudi Arabia.
authors.forename: Eman
authors.initials: E
authors.lastname: Al-Mussaed
authors.affiliation: Department of Pathology and Laboratory Medicine, Prince Sultan Military Medical City, Riyadh, Saudi Arabia.
authors.forename: Imad
authors.initials: I
authors.lastname: Elhag
authors.affiliation: Department of Pathology and Laboratory Medicine, Prince Sultan Military Medical City, Riyadh, Saudi Arabia.
authors.forename: Abdullah
authors.initials: A
authors.lastname: AlFiaar
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000032:analysis / Q000235:genetics; D002454:Cell Differentiation; D005260:Female; D019288:Herpesvirus 8, Human / Q000302:isolation & purification; D006801:Humans; D007121:Immunocompetence; D007150:Immunohistochemistry; D016130:Immunophenotyping; D017404:In Situ Hybridization, Fluorescence; D008297:Male; D008875:Middle Aged; D000069293:Plasmablastic Lymphoma / Q000737:chemistry / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology / Q000401:mortality / Q000473:pathology / Q000821:virology; D011237:Predictive Value of Tests; D016271:Proto-Oncogene Proteins c-myc / Q000235:genetics; D012307:Risk Factors; D012529:Saudi Arabia; D016019:Survival Analysis; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D014408:Biomarkers, Tumor; C489427:MYC protein, human; D016271:Proto-Oncogene Proteins c-myc