Orbital plasmablastic lymphoma--comparison of a newly reported entity with diffuse large B-cell lymphoma of the orbit.
OBJECTIVE
To describe two cases of orbital plasmablastic lymphoma (PBL), a recently defined aggressive large-cell lymphoma with a plasmacytic immunophenotype, typically occurring in the oral cavity of HIV+ patients. To compare their presentation, management, immunohistochemistry and prognosis with orbital diffuse large B-cell lymphoma (DLBCL).


METHODS
Consecutive, comparative case series of patients presenting to a specialist orbital service with histologically proven PBL or DLBCL.


RESULTS
Two cases of PBL and 14 cases of DLBCL were identified. The former were 40-49-year-old men, one HIV+, presenting with marked orbital/sinonasal tumour without oral involvement. Both tumours were negative for B-cell markers (CD20, CD79a), showed a plasmacytic immunophenotype (positive for CD 138, Vs38c), and displayed a Ki67 index of 100%. Despite chemotherapy, both patients died within 1 year. Of the 14 patients with DLBCL, 57% were female, mean age 65 yrs, and none were immunocompromised. Only 7 (50%) had sinus involvement and 3 (21%) had prior systemic lymphoma. All were positive for B-cell markers, with a Ki67 index of 55%-80%. None died within 1 year of diagnosis.


CONCLUSIONS
PBL can present in the orbit and is more aggressive than DLBCL. It must be considered in the differential of orbital large-cell lymphoid tumours showing plasmablastic morphology or immunophenotype.
Morley
Ana M S
AM
Moorfields Eye Hospital, London EC1V2PD, UK.
Verity
David H
DH
Meligonis
George
G
Rose
Geoffrey E
GE
D000328:Adult; D015703:Antigens, CD / Q000032:analysis; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D023241:Antiretroviral Therapy, Highly Active; D014408:Biomarkers, Tumor / Q000032:analysis; D003520:Cyclophosphamide / Q000627:therapeutic use; D003937:Diagnosis, Differential; D004317:Doxorubicin / Q000627:therapeutic use; D017809:Fatal Outcome; D015658:HIV Infections / Q000150:complications / Q000188:drug therapy; D006801:Humans; D016130:Immunophenotyping; D016393:Lymphoma, B-Cell / Q000188:drug therapy / Q000473:pathology*; D016403:Lymphoma, Large B-Cell, Diffuse / Q000188:drug therapy / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009918:Orbital Neoplasms / Q000188:drug therapy / Q000473:pathology*; D010950:Plasma Cells / Q000473:pathology*; D011241:Prednisone / Q000627:therapeutic use; D014057:Tomography, X-Ray Computed; D014750:Vincristine / Q000627:therapeutic use
D015703:Antigens, CD; D014408:Biomarkers, Tumor; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone