Diffuse Large B-cell Lymphomas With Plasmablastic Differentiation Represent a Heterogeneous Group of Disease Entities

Hematopathology Section, Laboratory of Pathology, and Department of Hematology, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi I Sunyer, University of Barcelona, Barcelona, Spain.
American Journal of Surgical Pathology (Impact Factor: 5.15). 07/2004; 28(6):736-47. DOI: 10.1097/01.pas.0000126781.87158.e3
Source: PubMed


Plasmablastic lymphoma was initially described as a variant of diffuse large B-cell lymphoma (DLBCL) involving the oral cavity of HIV+ patients and characterized by immunoblastic morphology and a plasma cell phenotype. However, other lymphomas may exhibit similar morphologic and immunophenotypic features. To determine the significance of plasmablastic differentiation in DLBCL and examine the heterogeneity of lymphomas with these characteristics, we examined 50 DLBCLs with low/absent CD20/CD79a and an immunophenotype indicative of terminal B-cell differentiation (MUM1/CD38/CD138/EMA-positive). We were able to define several distinct subgroups. Twenty-three tumors were classified as plasmablastic lymphoma of the oral mucosa type and showed a monomorphic population of immunoblasts with no or minimal plasmacytic differentiation. Most patients were HIV+ and EBV was positive in 74%. Eleven (48%) cases presented in the oral mucosa, but the remaining presented in other extranodal (39%) or nodal (13%) sites. Sixteen cases were classified as plasmablastic lymphoma with plasmacytic differentiation. These were composed predominantly of immunoblasts and plasmablasts, but in addition exhibited more differentiation to mature plasma cells. Only 33% were HIV+, EBV was detected in 62%, and 44% had nodal presentation. Nine cases, morphologically indistinguishable from the previous group, were secondary extramedullary plasmablastic tumors occurring in patients with prior or synchronous plasma cell neoplasms, classified as multiple myeloma in 7 of the 9. Two additional neoplasms were an HHV-8+ extracavitary variant of primary effusion lymphoma and an ALK+ DLBCL. HHV-8 was examined in 39 additional cases, and was negative in all. In conclusion, DLBCLs with plasmablastic differentiation are a heterogeneous group of neoplasms with different clinicopathological characteristics that may correspond to different entities.

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    • "Plasmablastic lymphoma is a rare and rapidly progressive variety of diffuse large B-cell lymphoma that was originally reported exclusively in the jaw and oral mucosa of male-predominant HIV-positive patients (Delecluse et al. 1997; Colomo et al. 2004; Yotsumoto et al. 2009). Its hallmarks include extensive local invasion, rapid dissemination and recalcitrance to treatment (Colomo et al. 2004; Scheper et al. 2005; Valenzuela et al. 2008). "
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    ABSTRACT: Plasmablastic lymphoma (PBL) is a rare and aggressive variant of diffuse large B cell lymphoma. The prognosis of PBL patients is poor. The majority of patients succumb to a fulminant disease course, with most dying in the first year after diagnosis. The small number of HIV-negative PBL cases reported in the literature to date is composed of single case reports and small case series. Consequently, the natural history of the disease in HIV-negative individuals and the optimum treatment are not well characterized. Intensive induction chemotherapy has been associated with marked improved overall survival. However the optimal regimen has not been defined. We describe the third case of PBL of the maxillary sinus which occurred in a 24-year old HIV-negative man. We outline the clinicopathological features and report success using a hyper-CVAD regimen with 6 cycles and consolidation radiation therapy yielding a complete remission of four years.
    Full-text · Article · Dec 2013 · SpringerPlus
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    • "However, this correlation may be imperfect, as[5], reported on case of ALK–DLBCL with NPM-ALK fusion that showed cytoplasmic ALK staining only. Thus, ALK gene rearrangements, originally thought to be uniquely associated with T-/null cell ALCL, have now been convincingly shown to occur in rare cases of B-cell lymphoma2345678910111213. Of note, prior to the initial series by[1,17]in 1996 reported NPM/ALK fusion transcripts (by RT PCR) in four of 33 cases of large B-cell lymphoma. "

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    • "Positive staining for plasma cell markers such as VS38c, CD38, MUM-1, and CD138 indicates a phenotype akin to plasma cells [8, 9]. Newer B-lineage markers (e.g., OCT.2 and BOB.1) may prove useful in determining a B-cell origin in plasmablastic lymphomas [10, 11]. "
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    ABSTRACT: Oral plasmablastic lymphoma (PBL) is a rare malignancy, associated with HIV or other immunocompromised conditions. The lesion constituted a new subtype of diffuse large B-cell lymphoma and proposed a distinct entity based on its basic morphology, its clinical behaviour involving predominantly extramedullary sites (particularly oral cavity), and its limited antigenic phenotype data suggesting plasmacytic differentiation. Authors here report a case of apparently healthy individual aged 35 years, presenting one-month history of swelling associated with loosened teeth around upper anteriors. Following incisional biopsy, routine histopathologic and immunohistochemical studies, the diagnosis of plasmablastic lymphoma was given.
    Full-text · Article · Sep 2012
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