Hematopoietic cell transplantation for plasmablastic lymphoma. Nonhodgkin lymphoma nhl is the sixth leadingcause of cancer death in the united states and accounts for 23% of primary neoplasms. We describe a case of plasmablastic lymphoma in an hiv infected patient who presented with an expanding oral lesion and symptoms of a toothache. Pblhiv, hivpositive patients with plasmablastic lymphoma. Most of these lymphomas are of bcell lineage and harbor clonal rearrangement of immunoglobulin genes. It is strongly associated with human immunodeficiency virus hiv infection and often occurs within the oral cavity. Plasmablastic lymphoma is a rare bcell lymphoma most often associated with hivaids or other forms of immunosuppression such as organ transplantation. Hiv associated pbl is an aidsdefining cancer, classified by who as a distinct. Although initially described as being associated with hiv, over the years it has also been identified in patients with solid organ transplant and.
Dezube3 plasmablastic lymphoma pbl is a distinct subtype of nonhodgkin bcell lymphoma, originally described with a strong predilection to the oral cavity of human immunode. Plasmablastic lymphoma in an hiv patient with cutaneous. Clinical characteristics and prognostic factors of plasmablastic lymphoma patients. Six years earlier, he had been diagnosed with hiv when he pre. Plasmablastic lymphoma pbl is a rare type of cancer most commonly found in hiv positive individuals. The pathogenesis of hivassociated lymphoma involves a complex interplay of biologic factors, such as chronic antigen stimulation, coinfecting oncogenic viruses, genetic abnormalities, and cytokine dysregulation. Hiv and plasmablastic lymphoma manifesting in sinus, testicles, and. Jun 02, 2016 plasmablastic lymphoma is strongly associated with hiv infection and other causes of immunodeficiency including organ transplantation and advanced age. Aidsassociated plasmablastic lymphoma presenting at the. Key points on hivassociated plasmablastic lymphoma hivassociated pbl is an aidsdefining cancer, classified by who as a distinct entity of aggressive dlbcl. Samuel winer and dariusz stachurski and kimberly perez and melhem. Pdf prognostic factors in chemotherapytreated patients.
Plasmablastic lymphoma pbl is an aggressive bcell malignancy that highly correlated with human immunodeficiency virus hiv. Plasmablastic lymphoma pbl is distinct type of diffuse large b cell lymphoma dlbcl predominantly seen in hiv positive patients. Pdf current knowledge on hivassociated plasmablastic lymphoma. Pbl was initially described in 1997, arising most commonly in the oropharyngeal area of hiv. Plasmablastic lymphoma pbl is an aggressive lymphoma commonly associated with hiv infection. Plasmablastic lymphoma pbl is an aggressive bcell malignancy highly associated with hiv. Bortezomibinduced tumor lysis syndrome in a patient with hiv.
Rare aidsassociated plasmablastic lymphoma as the initial. The biology and treatment of plasmablastic lymphoma blood. Apr 05, 2012 there are a number of welldefined genetic abnormalities in hiv associated lymphoma. Plasmablastic lymphoma pbl is a rare and aggressive subtype of nonhodgkin lymphoma that commonly occurs in human immunodeficiency virus hiv positive individuals, and affects oral sites. Patients with human immunodeficiency virus acquired immune deficiency syndrome hivaids are at increased risk for developing hodgkins lymphoma hl, a risk that has not decreased despite the success of combination antiretroviral therapy cart in the modern era.
Plasmablastic lymphoma is a unique aidsrelated nonhodgkin lymphoma nhl that presents as a rapidly growing destructive tumor, typically arising in the setting of a low cd4 count. Butera1,4 abstract plasmablastic lymphoma pbl is an aggressive variant of nonhodgkin lymphoma initially reported in the oral cavity of hiv. Plasmablastic lymphoma pbl is a rare lymphoma associated with immunosuppression. Plasmablastic lymphoma pbl is an aggressive form of nonhodgkins lymphoma nhl classically seen in patients infected with the human immunodeficiency virus, but can also be seen in other immunocompromised states such as transplant recipients, autoimmune diseases and the elderly. Nevertheless, pbl has also been described in hiv negative individuals, usually in association with other causes of immunosuppression steroid therapy for autoimmune disorders, solid organ transplantation, preexisting lymphoproliferative diseases or in elderly patients. The term plasmablastic lymphoma pbl refers to a rare and distinct entity classified by the world health organization who as an aggressive subtype of dlbcl, characterized by a diffuse proliferation of large neoplastic cells most of which resemble bcell immunoblasts, but in which tumor cells have a plasma cell immunophenotype. Winer,1,4 dariusz stachurski,1,3 kimberly perez,1,2 melhem jabbour,5 cannon milani,5 gerald a.
Lessons learned from 112 published cases plasmablastic lymphoma pbl is a distinct subtype of nonhodgkin bcell lymphoma, originally. Plasmablastic lymphoma is a rare bcell lymphoma most often associated with hiv aids or other forms of immunosuppression such as organ transplantation. A follicles are regressed with a paucity of lymphocytes, hypervascularity, and an indistinct border between the germinal center and surrounding mantle zone, which contains scattered, large, atypical. Prognostic factors in chemotherapytreated patients with hiv associated plasmablastic lymphoma. Key points on hiv associated plasmablastic lymphoma hiv associated pbl is an aidsdefining cancer, classified by who as a distinct entity of aggressive dlbcl. Plasmablastic lymphoma is a type of large bcell lymphoma, recognized in the who 2008 classification. Plasmablastic lymphoma is a rare, aggressive neoplasm that most commonly occurs in the setting of hiv infection and is associated with a dismal outcome in most. Hiv associated pbl is an aidsdefining cancer, classified by who as a distinct entity of aggressive dlbcl. Treating hiv associated lymphoma people with hiv infections are at increased risk for nonhodgkin lymphoma. Although people with hiv tend to get more aggressive forms of lymphoma such as diffuse large bcell lymphoma, primary cns lymphoma, or burkitt lymphoma, their outlook has improved a great deal in recent years. However, pbl can also be seen in patients with other immunodeficiencies as well as in immunocompetent individuals.
Aids associated plasmablastic lymphoma presenting at the insertion site of a peritoneal dialysis catheter a 37yearold man with known hiv disease and endstage renal disease esrd sought medical attention because of a persistent mass at the site of his peritoneal dialysis catheter. To date less than 250 cases have been published, of them 17 are pediatric. The diagnosis was based on histopathological examination and. 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 pbl is a distinct variant of diffuse large b.
Plasmablastic lymphoma is a rare and aggressive lymphoma that is strongly associated with hiv infection in its later stages. 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. Hivassociated hl hivhl differs from hl in nonhivinfected patients in that it is nearly always associated with epsteinbarr. Rare aidsassociated plasmablastic lymphoma as the initial presentation of aids. Lessons learned from 112 published cases jorge castillo,1 liron pantanowitz,2 and bruce j. Shriram jakate, md, frcpath, srinadh komanduri, md. Plasmablastic lymphoma of the oral cavity is a form of nonhodgkin lymphoma nhl and was first described in 1997. Pbl is generally associated with a poor prognosis despite chemotherapy. Plasmablastic lymphoma and related disorders american.
Here, we describe a case of plasmablastic lymphoma in a 50yearold female hivinfected patient. Differential diagnosis plasmablastic lymphoma surgical. Hivassociated pbl is an aidsdefining cancer, classified by who as a distinct. How i treat hivassociated lymphoma pubmed central pmc. Key points on hivassociated plasmablastic lymphoma hiv associated pbl is an aidsdefining cancer, classified by who as a distinct entity of aggressive dlbcl. Castillo 1 clinical department, hematology, national institute for infectious diseases lazzaro spallanzani rome, italy. Hivassociated plasmablastic lymphoma following haartrelated. Plasmablastic lymphoma pbl is a distinct subtype of non. Pathologically, pbl lacks expression of cd20, but because of its plasmacytic. There are a number of welldefined genetic abnormalities in hivassociated lymphoma.
The pathogenesis of this rare disease is related to immunodeficiency, chronic immune stimulation and ebv. 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. Current knowledge on hivassociated plasmablastic lymphoma. Current knowledge on hiv associated plasmablastic lymphoma. However, knowledge of the characteristic clinical setting, sites of involvement, and morphologic features of plasmablastic lymphoma can aid in the correct. In around 4 in 20 cases, it develops in the mouth, although it can affect other areas of the body. Clinical characteristics and prognostic factors of plasmablastic. Hivassociated plasmablastic castleman disease with concurrent nodal involvement by kaposi sarcoma. Pathology outlines plasmablastic lymphoma of oral mucosa type. Although pbl shares many morphologic and immunophenotypic features with multiple myeloma mm, it is classified by the world health organization as a subtype of diffuse large bcell lymphoma.
Current knowledge on hivassociated plasmablastic lymphoma michele bibas1 2and jorge j. Human immunodeficiency virus associated plasmablastic lymphoma. Pbl is a highly aggressive lymphoma strongly associated with immunosuppression, in particular with hiv infection. Improved outcome of human immunodeficiency virus associated plasmablastic lymphoma of the oral cavity in the era of highly active antiretroviral therapy. Plasmablastic lymphoma an overview sciencedirect topics. Bl is associated with activation of the myc gene and, when associated with hiv infection, resembles sporadic rather than endemic bl. A patient with plasmablastic lymphoma achieving longterm. There are a number of welldefined genetic abnormalities in hiv associated lymphoma table 1. Plasmablastic lymphoma pbl is a very rare form of dlbcl that is strongly associated with hiv, although it can develop in people with other immune system disorders. Because of its rarity, no standard management strategy has been established. Patients with hiv infection are also at higher risk of acquiring plasmablastic lymphoma. Plasmablastic myeloma versus plasmablastic lymphoma. Apr 09, 2015 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.
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