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Year : 2010  |  Volume : 53  |  Issue : 2  |  Page : 368-369
Plasmablastic lymphoma in a human immunodeficiency virus negative patient


Department of Pathology, Rajiv Gandhi Cancer Institute and Research Centre, Sector-5, Rohini, Delhi - 110 085, India

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Date of Web Publication12-Jun-2010
 

How to cite this article:
Khurana A, Jalpota Y. Plasmablastic lymphoma in a human immunodeficiency virus negative patient. Indian J Pathol Microbiol 2010;53:368-9

How to cite this URL:
Khurana A, Jalpota Y. Plasmablastic lymphoma in a human immunodeficiency virus negative patient. Indian J Pathol Microbiol [serial online] 2010 [cited 2019 Nov 21];53:368-9. Available from: http://www.ijpmonline.org/text.asp?2010/53/2/368/64281


We discuss a case of 55-year-old male who presented with facial asymmetry two weeks prior to presentation. He also complained of dysphagia along with a mass in the right posterior cervical region. No other regional lymphadenopathy and hepatosplenomegaly were noted. A magnetic resonance imaging (MRI) of neck region revealed a bulky mass comprising of multiple confluent lymph nodes in the right posterior cervical region. Biopsy of the mass showed a high grade tumor comprising of diffuse sheets of pleomorphic cells having oval vesicular nuclei, prominent nucleoli and scant amphophilic cytoplasm with an overall morphology resembling immunoblasts [Figure 1]. The mitotic activity was very brisk, and starry sky pattern could be appreciated in a few areas. On immunohistochemistry (IHC), the tumor cells were positive for vimentin, epithelial membrane antigen (EMA) and expressed CD79a focally. Pan-cytokeratin, leukocyte common antigen (LCA), S-100, other B and T cell markers including CD20, ALK and Tdt were negative. Ki67 index was nearly 80% revealing a high proliferation fraction [Figure 2]. The tumor cells expressed CD38, CD138 and demonstrated kappa (κ) light chain restriction [Figure 3]. The blastic morphology and plasma cell-like immunophenotype features helped establish a diagnosis of plasmablastic lymphoma. A bone marrow biopsy showed neoplastic infiltration. A whole body positron emission tomography (PET) scan showed an extensive metastatic disease. The disease was designated as stage IV. The serum protein electrophoresis and serum-ionized calcium levels were normal and no lytic bone lesions were seen on radiology. Serology was negative for human immunodeficiency virus (HIV), HBsAg and hepatitis C virus (HCV) infection. The patient was started on CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone) based chemotherapy. Post 3 cycles a re-evaluation MRI was done which showed a near complete regression in the primary nodal mass.


   Discussion Top


Plasmablastic lymphoma (PBL) is an uncommon malignancy, described almost exclusively as an acquired immunodeficiency syndrome (AIDS) related lymphoma. [1] The recent WHO classification of lymphoid neoplasms addresses plasmablastic lymphoma (PBL) as a separate entity under mature B cell neoplasms, unlike previously being discussed as a variant of diffuse large B cell lymphoma (DLBCL). [1] Classically, the patient presents at an advanced stage with extranodal site involvement, usually oral cavity and jaw. [1],[2] It is an aggressive neoplasm with most patients dying in the first year of life. [1],[3] The occurrence of this lymphoma in a HIV negative individual is further less common. However, over the years its occurrence in HIV negative individuals has been noticed. It has been reported as a lymphoproliferative disorder in immunocompromised HIV negative individuals, who have undergone transplant procedures and developed PBL later on. [4] Plasmablastic lymphoma has been reported in HIV negative patients at unusual sites like visceral cranium, cervix and thorax. [5] Epstein-Barr virus infection has shown association in both HIV positive as well as HIV negative patients. [1] Nodal involvement is uncommon, and is a feature commonly noted in HIV negative cases. Plasmablastic plasma cell myeloma and diffuse large B cell lymphoma (DLBCL) with plasmacytic differentiation are close histological differentials to PBL. The cytomorphology and immunophenotype of PBL is virtually identical to plasmablastic plasma cell myeloma and hence clinical correlation is required to distinguish these two entities. Absence of serum monoclonal protein with no radiological evidence of lytic bone lesions favors PBL. [2] Leukocyte common antigen (LCA) and CD20 positivity uniformly seen in DLBCL cases is usually weak to scant in PBL. Other morphological differential diagnoses, which can be ruled out based on IHC are Burkitt lymphoma, lymphoblastic lymphoma, poorly differentiated carcinoma and malignant melanoma.

Currently no standard treatment protocol is recommended. Highly active antiretroviral therapy (HAART) in combination with CHOP, CVAD etc. has been used in HIV positive cases. The indexed case showed excellent response to CHOP regimen.

Though the outcome of this rare lymphoma is poor, the overall survival is better in HIV positive cases put on HAART as compared to HIV negative patients. To conclude, the diagnosis of PBL should be considered in HIV negative individuals as this aggressive neoplasm warrants an early diagnosis and therapy.

 
   References Top

1.Stein H, Harris NL, Campo E. Mature B-cell neoplasms. In: WHO classification of tumours of haematopoeitic and lymphoid tissues. Lyon:IARC Press; 2008. p. 256-7.  Back to cited text no. 1      
2.Vega F, Chang CC, Medeiros LJ, Udden MM, Cho-Vega JH, Lau CC, et al. Plasmablastic lymphomas and plasmablastic plasma cell myelomas have nearly identical immunophenotypic profiles. Mod Pathol 2005;18:806-15.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]  
3.Teruya-Feldstein J, Chiao E, Filippa DA, Lin O, Comenzo R, Coleman M, et al. CD20-negative large-cell lymphoma with plasmablastic features: A clinically heterogenous spectrum in both HIV-positive and -negative patients. Ann Oncol 2004;15:1673-9.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]  
4.Borenstein J, Pezzella F, Gatter KC. Plasmablastic lymphomas may occur as post-transplant lymphoproliferative disorders. Histopathology 2007;51:774-7.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]  
5.Masgala A, Christopoulos C, Giannakou N, Boukis H, Papadaki T, Anevlavis E. Plasmablastic lymphoma of visceral cranium, cervix and thorax in an HIV-negative woman. Ann Hematol 2007;86:615-8.  Back to cited text no. 5  [PUBMED]  [FULLTEXT]  

Top
Correspondence Address:
Anuj Khurana
98 SFS Flats, Phase-4, Ashok Vihar, Delhi - 110 052
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.64281

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    Figures

  [Figure 1], [Figure 2], [Figure 3]

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