This suggests an important role of ebv in the pathogenesis. The biology and treatment of plasmablastic lymphoma blood. Plasmablastic lymphoma pbl is an aggressive lymphoma commonly. Because of its rarity and lack of expression of markers used in establishing an initial hematopoietic lineage, it poses. A patient with plasmablastic lymphoma achieving longterm.
A case of plasmablastic lymphoma achieving complete response. Pdf plasmablastic lymphoma pbl is an aggressive subtype of nonhodgkins. How i treat hiv associated lymphoma kieron dunleavy. Plasmablastic lymphoma occurs predominantly in patients with advanced hiv disease, although it has also been reported in hiv negative individuals. Plasmablastic lymphoma pbl is a rare and aggressive subtype of non hodgkin lymphoma that commonly occurs in human immunodeficiency virus hiv positive individuals, and affects oral sites. Hodgkin lymphoma that was once believed to occur primarily in the oral cavity of human immunodeficiency virus. Numerous extraoral sites have also been reported to date. Plasmablastic lymphoma pbl is a rare and aggressive bcell malignancy with. Plasmablastic lymphoma pbl is a distinct subtype of non. Epidemiologically, hivpositive patients have a 60 to 200fold increased incidence of nonhodgkin lymphoma nhl, the majority of which are dlbcl. Plasmablastic lymphoma pbl is a distinct subtype of non hodgkin bcell lymphoma, originally described with a strong predilection to the oral cavity of human immunodeficiency virus hiv infected. Hivassociated lymphomas and gammaherpesviruses blood.
Although the condition most commonly occurs in the oral cavity, it can be diagnosed in many other parts of the body such as the gastrointestinal tract, lymph nodes, and skin. Page 3 of 6 oe e 00 3 e a oe ae oa 25563 immunoblastic and plasmablastic morphology with. Plasmablastic lymphoma is a rare bcell lymphoma most often associated with hivaids or other forms of immunosuppression such as organ transplantation. Plasmablastic myeloma versus plasmablastic lymphoma. Plasmablastic lymphoma was initially reported as an hiv associated lymphoplasmacytoid malignancy 28. Hiv and plasmablastic lymphoma manifesting in sinus. Plasmablastic lymphoma pbl is an aggressive variant of non hodgkin lymphoma initially reported in the oral cavity of hiv positive individuals. Extraoral, hiv, non hodgkins lymphomas, plasmablastic introduction plasmablastic lymphoma pbl is classified as a distinct entity by the world health organization and typically occurs in the oral cavity in the clinical setting of hiv infection, accounting for 2. As haart improves, prognosis is defined more by tumorrelated features and less by hiv. Im sorry to hear that you have been diagnosed with non hodgkin plasmablastic lymphoma. Plasmablastic lymphoma of the oral cavity is a form of non hodgkin lymphoma nhl and was first described in 1997. The cell of origin involved in plasmablastic lymphoma pbl is the plasmablast. We report here a human immunodeficiency virus infected patient surviving over 12 years following treatment for advanced pbl with epoch chemotherapy and intrathecal.
This makes it harder for your body to fight infections. Rare aidsassociated plasmablastic lymphoma as the initial. Kaposi sarcoma and hivassociated lymphoproliferative. The current belief is that the plasmablast is derived from a postgerminal center bcell that has undergone preterminal differentiation via somatic hypermutation and class switching recombination to resemble a plasma cell rather than a bcell castillo et al. Differentiating between diffuse large b cell lymphoma dlbcl with plasmablastic differentiation and true cases of plasmablastic lymphoma pbl is sometimes challenging, particularly as a substantial overlap in immunphenotype exists between late. Without these t cells, other cells in your immune system, such as b cells, cant work properly. We describe a case of plasmablastic lymphoma in an hiv infected patient who presented with an expanding oral lesion and symptoms of a toothache. In parallel, some cases of extraoral pbl have also been observed in hivnegative patients. No standard of care is established for this disease and prognosis remains extremely poor. Hiv and non hodgkin lymphoma university of washington.
Investigators reported a patient with hiv related non hodgkins lymphoma originating in the oral cavity. Human immunodeficiency virus related non hodgkin lymphoma. Plasmablastic lymphoma pbl is a recently recognized aggressive non hodgkins bcell lymphoma which occurs predominantly in hiv seropositive individuals and shows a predilection for the oral cavity. However, pbl is also seen in other immunodeficient and immunocompetent individuals. Recently the world health organization who has recognized plasmablastic lymphoma as a different entity under mature bcell neoplasm 1, 2. There is some information on our website on non hodgkin lymphoma which might be of interest, to read more about it please click here also, our cancer nurses are just a phone call away, if you need to talk to someone on the phone. This report details a case of the recently described entity plasmablastic lymphoma of the mouth in a patient who was later found to have severe hiv disease. Plasmablastic lymphoma pbl is a rare lymphoma associated with immunosuppression. Plasmablastic lymphoma in an hiv patient with cutaneous. Plasmablastic lymphoma pbl is a rare aggressive subtype of b cell lymphoma commonly associated with hiv infection. Plasmablastic lymphoma and related disorders eric d. Plasmablastic lymphoma pbl is an aggressive subtype of nonhodgkins lymphoma nhl, which frequently arises in the oral cavity of human immunodeficiency virus hiv infected patients. Background plasmablastic lymphoma pbl is a rare and aggressive subtype of non hodgkin diffuse large bcell lymphoma originally with a predilection to the oral cavity of patients infected with hiv.
Plasmablastic lymphoma pbl is a rare and highly aggressive variant of. The histologic features are those of mccd, but plasmablasts coalesce within follicle centers. Plasmablastic lymphoma pbl is an uncommon, highly aggressive type of lymphoma, which classically has been described with the highest incidence in hiv positive individuals typically presenting. Plasmablastic lymphoma pbl is a rare, aggressive lymphoma of bcell lineage and is frequently but not always associated with hiv and epsteinbarr virus ebv infection. Plasmablastic lymphomanonhodgkins lymphomabcell lymphomaaidsrelated lymphomalenalidomide.
Plasmablastic lymphoma pbl is an aggressive bcell malignancy highly associated with hiv. Plasmablastic lymphoma is an aggressive form of non hodgkin lymphoma. We report a patient with hivnegative pbl who achieved. The prevalence of hivpositive and hivnegative cases is not. Plasmablastic lymphoma of the maxillary sinus in an hiv. The biology and treatment of plasmablastic lymphoma. Hiv associated lymphomas aidsdefining lymphomas are defined as aggressive bcell non hodgkins lymphomas arising in patients who have hiv burkitts diffuse large bcell primary cns plasmablastic primary effusion lymphoma hiv also increases the risk of classic hodgkin lymphoma. Pbl is more commonly associated with preceding im munodeficiency and accounts for 2. Later reports found that pbl also occurs in nonhiv patients and can manifest in. Plasmablastic lymphoma an overview sciencedirect topics.
Plasmablastic lymphoma pbl is a rare and highly aggressive variant of diffuse large b cell lymphoma with median survival of advanced stage patients varying between 6 and 15 months in previous reports. Plasmablastic lymphoma pbl is an aggressive sub type of non hodgkin s lymphoma nhl, which frequently arises in the oral cavity of human imm unode ciency virus hiv infected patients. Plasmablastic lymphoma pbl is a rare type of cancer most commonly found in hiv positive individuals. Plasmablastic lymphoma pbl is a clinicopathological entity that was initially described in 1997 1 and is now considered a distinct subtype of diffuse large bcell lymphoma dlbcl seen more commonly in patients with hiv infection. Plasmablastic lymphoma pbl is a challenging diagnosis given its rarity and lack of expression of markers that are usually used by pathologists in establishing hematopoietic lineage. Plasmablastic lymphoma versus diffuse large b cell. Unlike diffuse large bcell lymphoma, the most common aggressive lymphoma, pbl is associated with a dire prognosis when treated using regimens commonly adopted for diffuse. Non hodgkins lymphoma is the second most common hiv associated malignancy.
Nevertheless, pbl has also been described in hivnegative individuals, usually in association with other causes of immunosuppression steroid. The tumor cells were characteristic in that they were negative for. Non hodgkin lymphoma nhl is the sixth leadingcause of cancer death in the united states and accounts for 23% of primary neoplasms. To the authors knowledge, however, only 3 reports in the literature describe its cytologic features. Highgrade non hodgkins lymphomas with plasmablastic differentiation are frequently encountered in hiv positive patients. Because of its rarity, no standard management strategy has been established. An ongoing study sponsored by the city of hope medical center is examining the feasibility and safety of gene therapy that uses recombinant rna to target a key element in the hiv genome in patients who have hiv aids and a non hodgkins lymphoma, including patients with plasmablastic lymphoma.
Positive plasmablastic lymphoma lanfang miao state key laboratory of oncology in south china, collaborative innovation center for cancer medicine, guangzhou, guangdong, 510060 china. Since its original description, several cases have been reported in patients who do not have hiv infection. Plasmablastic lymphoma is strongly associated with hiv. Oral plasmablastic lymphoma in previously undiagnosed hiv. Plasmablastic lymphoma pbl is an aggressive variant of nonhodgkin lymphoma initially reported in the oral cavity of hivpositive individuals. However, knowledge of the characteristic clinical setting, sites of involvement, and morphologic features of plasmablastic lymphoma can aid in the correct. Hivpositive cases and 50% of the hivnegative cases. It is strongly associated with human immunodeficiency virus hiv infection and often occurs within the oral cavity. Plasmablastic lymphoma pbl is distinct type of diffuse large b cell lymphoma dlbcl predominantly seen in hiv positive patients. Plasmablastic lymphoma and related disorders american. Lymphomas occurring specifically in hiv positive patients include primary effusion lymphoma pel and its solid variants, plasmablastic lymphoma of the oral cavity type and large bcell lymphoma arising in kaposi sarcoma herpesvirus kshvassociated multicentric castleman disease. Plasmablastic lymphoma pbl is an aggressive subtype of nonhodgkins lymphoma nhl, which frequently arises in the oral cavity of human immunodeficiency virus hiv.
1198 20 872 1109 223 526 233 760 1453 107 233 1431 396 63 920 1025 178 1264 374 1211 171 1161 1168 156 1084 412 723 1010 1237 969 795 978 391 15 399 37 1095 1151