Plasmablastic lymphoma in a previously undiagnosed AIDS patient: A case report.

Nagpal D, Maralingannavar M, Koshti S. 
 Vieira FO, El Gandour O, Buadi FK, Williams GB, Shires CB, Zafar N. Plasmablastic lymphoma in a previously undiagnosed AIDS patient: A case report. Head and Neck Pathol 2008;2:92-96.

Sarode SC, Sarode GS, Patil A. Plasmablastic lymphoma of the oral cavity: A review. Oral Oncol 2010;46:146-153.

Vieira F, O. et al, 2008

Nagpal D et al, 2010

The epidemiology and prognosis of AIDS has changed tremendously since the first description of disease in 1980 [1]. The introduction of effective therapy and prophylaxis for opportunistic infections has significantly reduced mortality [2, 3]. Highly active antiretroviral therapy (HAART), introduced in 1996, has not only reduced the complications associated with AIDS, but also significantly improved survival [4–6]. However, secondary malignancies continue to be a major cause of AIDS-related morbidity and mortality [5]. Recent reports have shown a significant decrease in the incidence of non-Hodgkin lymphoma (NHL) in AIDS patients on HAART [7, 8], but the incidence still remains much higher than that of the general population [3, 9, 10]. The prevalence of AIDS-related malignancies is expected to increase as AIDS patients continue to live longer [4, 5, 8]. Therefore, PBL is expected to be seen more frequently among the spectrum of AIDS-defining illness [11].

Since its first description in early 1980’s AIDS epidemiology and prognosis has changed drastically due to the effective use of HAART1. After its introduction in 1996 HAART has been demonstrating success in reducing the complications associated with AIDS thus improving the survival. But secondary malignancies are still of concern for the AIDS related morbidity and mortality 2.

Sarode SC et al, 2010	Nagpal D et al, 2010
As per the current view, PBL arising from a post-germinal centre, preterminally differentiated B-cell lineage shows an abrupt dif ferentiation arrest in the plasmablastic stage.20 Oral PBL effaces the mucosal architecture with infiltration of the submucosa and extension to the mucosal surface with ulceration. It is characterised by monomorphic cellular proliferation of large lymphoid cells in a diffuse sheet-like and cohesive growth pattern. Thus as in other forms of NHL, it is likely that EBV has an important role in the pathogenesis of oral PBL, but a similar role for HHV8 is less certain.	PBL arising from post-germinal, preterminally differentiated B-cell lineage with abrupt arrest in the Plasmablastic stage. It is characterized by monomorphic cellular proliferation of large lymphoid cells in a diffuse cohesive or sheet-like pattern 5. Recent reviews have described that EBV has an important role in the pathogenesis of oral PBL, but a similar role for HHV8 is less certain 6.
Vieira FO et al, 2008	Nagpal D et al, 2010
The natural history of PBL without adequate treatment is progression from local to systemic disease and subsequent death.	The natural history of PBL without adequate treatment is progression from local to systemic disease and subsequent death 2.
Vieira FO et al, 2008	Nagpal D et al, 2010
Discussion The World Health Organization (WHO) classifies PBL as a non-Hodgkin B-cell lymphoma, predominantly occurring in HIV-positive patients. Originally PBL was described in the oral cavity region although, many reports of patients without HIV and outside the head and neck area have been recently presented in the literature [10, 13, 16–19]. PBL presents an aggressive behavior mostly in immunosuppressed HIV-positive patients who are co-infected by	Discussion The World Health Organization (WHO) classifies PBL as a non-Hodgkin B-cell lymphoma, predominantly occurring in HIV-positive patients. Originally PBL was described in the oral cavity region although; many reports of patients without HIV and outside the head and neck area have been recently presented in the literature 7-10. PBL has an aggressive nature especially in immunicompromised HIV-positive patients. Once antiretroviral therapy is initiated a

EBV. Interestingly, once antiretroviral therapy is initiated a more favorable and indolent response is observed.	more favorable and indolent response is observed 11.
Sarode SC et al, 2010	Nagpal D, 2010
Oral PBL effaces the mucosal architecture with infiltration of the submucosa and extension to the mucosal surface with ulceration. It is characterised by monomorphic cellular proliferation of large lymphoid cells in a diffuse sheet-like and cohesive growth pattern. The sheets of tumor cells are interspersed with macrophages, resulting in a ‘starry-sky’ appearance on low power examination. The tumor cells are reminiscent of immunoblasts and plasmablastic differentiation in the form of a round to oval shape with either centrally or eccentrically placed nuclei and abundant eosinophilic. cytoplasm (Fig. 2). Dutcher and Russell bodies characteristic of plasma cells are not seen.4 Usually, a single prominent nucleoli is located in the center of the nucleus. Brisk mitotic index, apoptotic bodies and sometimes necrosis has also been reported.	On light microscopy oral PBL shows infiltration of submucosa and extension to mucosal surface with ulceration, this was not found in the present case. It is characterized by monomorphic cellular proliferation of large lymphoid cells in diffuse sheet like and cohesive growth pattern. The sheets of tumor cells are interspersed with macrophages resulting in a ‘starry-sky’ appearance on low power examination. The tumor cells are reminiscent of immunoblasts and Plasmablastic differentiation in the form of round to oval shape with either centrally or eccentrically placed nuclei and abundant eosinophilic cytoplasm. Usually, single prominent nucleolus is located in the center of the nucleus. Brisk mitotic index, apoptotic bodies and sometimes necrosis has also been reported 5.
Vieira FO et al, 2008	Nagpal D et al, 2010
Once PBL is confirmed, work-up tests for HIV should be included due to this strong association [4, 6, 11–13, 20– 22]. In case the test results confirmed PBL-AIDS associated, there is evidence that initiating HAART improves prognosis, decreases incidence of systemic complications, and improves survival [4, 14, 19, 20, 23].	Once PBL is confirmed, work-up tests for HIV should be included due to this strong association 12,13 . In case the test results confirmed PBL-AIDS association, there is evidence that initiating HAART improves prognosis, decreases incidence of systemic complications, and improves survival 4, 10, 14-16.
Sarode SC et al, 2010	Nagpal D et al, 2010
Treatment may consist of chemotherapy, using prednisolone, cyclophosphamide, adriamycin and/or vincristine, or local excision followed by radiation. The final course of therapy is considered on a case-by-case basis depending on the stage of the disease, the presence of systemic symptoms or the association with HIV infection.7 Local radiotherapy has proven successful	Treatment may consist of chemotherapy, local excision followed by radiation. The final course of therapy is considered on case-by-case basis depending on the stage of the disease, the presence of systemic symptoms or the association with HIV infection. Local radiotherapy has proven successful only on gingival lesions. Multidrug chemotherapy with or without radiation therapy is

when only gingival lesions are present. Multi-drug chemotherapy with or without radiation treatment is generally recommended for the disseminated disease.	recommended for disseminated disease 17.
Vieira FO et al, 2008	Nagpal D et al, 2010
Currently there is agreement that HIV-positive patients with PBL must commence HAART [14, 19, 20, 23, 24], since it has shown improvement in survival rates.	Currently there is agreement that HIV-positive patients with PBL must commence HAART since it has shown to improve the survival rate 2,5,14.
Sarode SC et al, 2010	Nagpal D et al, 2010
Conclusion Oral PBL is an uncommon, recently described B-cell derived lymphoma most commonly seen in patients with HIV infection. It is characterised by a diagnostic triad of predilection for gingivo-buccal complex mucosa, classical plasmablastic morphology with the lack of neoplastic plasma cells and a limited immunohistochemical panel consisting of CD20 negativity, LCA (+/_), CD138/ VS38c diffuse positivity, light chain restriction and high Mib-1 index.	Conclusion Oral PBL is not uncommon among HIV-positive individuals, classified under non-hodgkin’s type of lymphoma derived from the B cells 1,2. It is characterized by a diagnostic triad of predilection for gingiva-buccal complex mucosa, classical Plasmablastic morphology with lack of neoplastic plasma cells and limited immunohistochemical panel consisting of CD20 negativity, LCA (+/-), CD138 positivity and high Mib-1 index 5.

Plasmablastic lymphoma in a previously undiagnosed AIDS patient: A case report. International Journal of Contemporary Dentistry 2010;1(3):7-10.