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Query: UMLS:C0026764 (multiple myeloma)
 36,148 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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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PMID:Diffuse large B-cell lymphomas with plasmablastic differentiation represent a heterogeneous group of disease entities. 1516 65

Plasmablastic lymphoma is an aggressive neoplasm that shares many cytomorphologic and immunophenotypic features with plasmablastic plasma cell myeloma. However, plasmablastic lymphoma is listed in the World Health Organization (WHO) classification as a variant of diffuse large B-cell lymphoma. To characterize the relationship between plasmablastic lymphoma and plasmablastic plasma cell myeloma, we performed immunohistochemistry using a large panel of B-cell and plasma cell markers on nine cases of plasmablastic lymphoma and seven cases of plasmablastic plasma cell myeloma with and without HIV/AIDS. The expression profiles of the tumor suppressor genes p53, p16, and p27, and the presence of Epstein-Barr virus (EBV) and human herpes virus type 8 (HHV-8) were also analyzed. All cases of plasmablastic lymphoma and plasmablastic plasma cell myeloma were positive for MUM1/IRF4, CD138, and CD38, and negative for CD20, corresponding to a plasma cell immunophenotype. PAX-5 and BCL-6 were weakly positive in 2/9 and 1/5 plasmablastic lymphomas, and negative in all plasmablastic plasma cell myelomas. Three markers that are often aberrantly expressed in cases of plasma cell myelomas, CD56, CD4 and CD10, were positive in 5/9, 2/5, and 6/9 plasmablastic lymphomas, and in 3/7, 1/5, and 2/7 plasmablastic plasma cell myelomas. A high Ki-67 proliferation index, overexpression of p53, and loss of expression of p16 and p27 were present in both tumors. No evidence of HHV-8 infection was detected in either neoplasm. The only significant difference between plasmablastic lymphoma and plasma cell myeloma was the presence of EBV-encoded RNA, which was positive in all plasmablastic lymphoma cases tested and negative in all plasma cell myelomas. In conclusion, most cases of AIDS-related plasmablastic lymphoma have an immunophenotype and tumor suppressor gene expression profile virtually identical to plasmablastic plasma cell myeloma, and unlike diffuse large B-cell lymphoma. These results do not support the suggestion in the WHO classification that plasmablastic lymphoma is a variant of diffuse large B-cell lymphoma.
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PMID:Plasmablastic lymphomas and plasmablastic plasma cell myelomas have nearly identical immunophenotypic profiles. 1557 69

Plasmablastic lymphoma is an aggressive diffuse large B-cell lymphoma classically arising in the oral cavities and jaws of individuals infected with the human immunodeficiency virus (HIV). More recently, cases of plasmablastic lymphoma have been identified in individuals negative for HIV. We report a case of plasmablastic lymphoma presenting as a rapidly expanding upper extremity mass in a 66-year-old individual negative for HIV. Aggressive multiple-agent chemotherapy resulted in a dramatic improvement of all symptoms. Increasing awareness of plasmablastic lymphoma in individuals who are HIV negative can allow for a better understanding of its clinical course and for specific chemotherapeutic regimens to be developed.
Clin Lymphoma Myeloma 2006 May
PMID:Plasmablastic lymphoma presenting as an arm mass in an individual negative for human immunodeficiency virus: a case report. 1679 82

Plasmablastic lymphoma, which is considered a subtype of diffuse large B-cell lymphoma, shares many similar morphological and immunophenotypic features with plasmablastic transformation of plasma cell myeloma. In the setting of human immunodeficiency virus (HIV) infection, both types of neoplasms can be associated with Epstein-Barr virus (EBV), thus making their distinction challenging. Moreover, the biological relationship between these entities remains unclear. We report four unique cases of plasmablastic lymphoma occurring in the setting of HIV infection that had overlapping clinical and genetic features with plasma cell myeloma. We reviewed the clinical, morphological, and cytogenetic findings and performed immunohistochemistry, in situ hybridization for EBV, chromosome analysis, and fluorescent in situ hybridization (FISH) using the MYC break-apart rearrangement probe. All patients were males with a median age of 45 years. In addition to extra-nodal disease, plasmablastic morphology, and phenotype typical of plasmablastic lymphoma, three of the four cases also showed clinical findings overlapping with plasma cell myeloma, that is, monoclonal serum immunoglobulin and lytic bone lesions. Furthermore, these cases showed complex cytogenetic changes that are more commonly observed in plasma cell myeloma. A unique feature was the presence of MYC (8q24.1) rearrangement confirmed by FISH in all four cases. MYC translocation has been associated with tumor progression in multiple myeloma but has only rarely been previously reported in plasmablastic lymphoma. These cases show a clinical and biological relationship between plasmablastic lymphoma and the plasmablastic variant of plasma cell myeloma. Dysregulation of MYC may be a common genetic mechanism that imparts plasmablastic morphology and aggressive clinical course to B-cell neoplasms at a later stage of differentiation.
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PMID:Plasmablastic lymphoma with MYC translocation: evidence for a common pathway in the generation of plasmablastic features. 2034 82

Plasmablastic lymphoma (PBL) is an uncommon aggressive lymphoma arising most frequently in the oral cavity of HIV-infected patients. Rare cases of PBL have been reported in extraoral sites, particularly extranodal sites, as well as in immunocompetent patients. We report an unusual case of PBL in a 69-year-old, HIV-negative non-immunocompromised man presenting with generalized lymphadenopathy. To our knowledge, this is the first case of PBL presented as primarily generalized lymphadenopathy in HIV-negative patients. Histologic examinations of cervical, inguinal and axillary lymph nodes demonstrated a neoplastic proliferation of large cells with extensive necrosis. The neoplastic cells formed sheets with a relatively cohesive growth pattern interspersed by small lymphocytes and plasma cells. The large tumor cells expressed MUM1, OCT-2 and BOB.1, and were negative for CD138, CD38, AE1/AE3, melan A, PLAP, S100, vimentin, CD117, CD30, ALK-1, leukocyte common antigen (CD45), T-cell, B-cell and histolytic markers, CD56, CD10 and BCL-6. The proliferation index by Ki-67 immunohistochemistry was approaching 100%. In situ hybridization for Epstein-Barr Virus-encoded RNA (EBER) was positive in large malignant cells. A diagnosis of PBL was made. These findings indicate that PBL should be included in the differential diagnosis of an HIV-negative, immunocompetent patient with generalized lymphadenopathy. The adjacent plasma cells were positive for CD138 and CD38 and show kappa-light chain restriction, but without EBER expression, raising the possibility of a preexisting or concurrent plasmacytoma and that the PBL may be a high-grade transformation from a preexisting plasma cell neoplasm following Epstein-Barr virus infection. Electron microscopy showed numerous circumferential long slender peripheral cytoplasmic projections in the large tumor cells, suggesting that some of the previously reported large B-cell lymphoma with cytoplasmic projections may actually be PBL.
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PMID:Plasmablastic lymphoma may occur as a high-grade transformation from plasmacytoma. 2096 60

Plasmablastic lymphoma (PBL) is an aggressive variant of non-Hodgkin lymphoma initially reported in the oral cavity of HIV-positive individuals. Since its original description, several cases have been reported in patients who do not have HIV infection. However, despite its recognition as a distinct subtype of diffuse large B-cell lymphoma several years ago, comprehensive reviews of this entity are lacking. A MEDLINE search through June 2010 was performed to identify cases with a pathologic diagnosis of HIV-negative PBL based on morphology and minimal immunohistochemical criteria. Our study included a total of 76 cases. The median age was 57 years (range, 1 to 90 years) with a male-to-female ratio of 1.7. Seventy-four percent of cases did not have an apparent association with immunosuppression, 18% had a concurrent lymphoproliferative or autoimmune disorder and 9% developed PBL after solid organ transplantation. Oral involvement was observed in 21%, advanced stage in 60%, Epstein-Barr virus-encoded RNA expression was positive in 45% and Ki-67 expression of greater than or equal to 80% in 61% of the cases. Chemotherapy was documented in 43 patients, from which 43% received the cyclophosphamide, doxorubicin, vincristine, prednisone (CHOP)-like regimens. The median and the 2-year overall survival for the whole group were 9 months and 10%, respectively. Patients who had HIV-negative PBL have distinct clinicopathological characteristics, such as short overall survival and lower rates of oral involvement and Epstein-Barr virus-encoded RNA expression than the previously reported in HIV-positive patients.
Clin Lymphoma Myeloma Leuk 2011 Apr
PMID:HIV-negative plasmablastic lymphoma: not in the mouth. 2157 22

Plasmablastic lymphoma (PBL) of bone is a rare neoplasm that shares many confusing cytomorphological and immunohistochemical features with plasmablastic plasma cell myeloma (PBPCM). A 47-year-old female patient presented with a bony swelling and bone pain in the left humerus for the last 6 months. On radiological examination (x-ray and computed tomography) it appeared to be a lytic lesion, and a pathological fracture was detected. The patient was HIV-negative. Fine needle aspiration (FNA) was done from the lesion, which was inconclusive. Subsequently, incisional biopsy was taken. Histopathological examination and immunohistochemistry confirmed a high-grade plasmablastic neoplasm, favoring a diagnosis of PBL. Most of the reported cases of PBL have occurred in HIV-positive patients, and the bone is a very rare site. PBL can be confused with PBPCM. A final diagnosis should be rendered only after thorough histopathological and immunohistochemical examination.
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PMID:High-grade plasmablastic neoplasm of humerus in an HIV-negative patient, which was indeterminate between plasmablastic lymphoma and plasmablastic myeloma. 2176 19

Plasmablastic lymphoma (PBL) is an aggressive lymphoma classified by the World Health Organization as a subtype of diffuse large B-cell lymphoma that shares many morphologic and immunophenotypic features with multiple myeloma. It is extremely rare in immunocompetent patients. Because of the small number of patients reported, this rare lymphoma remains a poorly characterized and understood entity with presently no standard recommendations regarding the optimal treatment. Herein, we report a dramatic clinical response coupled with tumor lysis syndrome to a bortezomib-based treatment in an HIV-negative patient with refractory plasmablastic lymphoma.
Clin Lymphoma Myeloma Leuk 2010 Oct
PMID:Bortezomib-induced tumor lysis syndrome in a patient with HIV-negative plasmablastic lymphoma. 2185 50

Plasmablastic lymphoma (PBL) is an aggressive diffuse large B-cell lymphoma (DLBCL) with plasmablastic features that was initially described in the oral cavity of HIV-infected individuals. PBL remains a diagnostic challenge given its close morphologic resemblance and overlapping immunophenotypic patterns to other B-cell lymphoid malignancies and plasmablastic plasma cell myeloma (PCM) with extramedullary involvement. The presence of serum monoclonal protein and radiographic evidence of lytic bone lesions favors the diagnosis of plasma cell myeloma over PBL. Distinguishing PBL from PCM is important as PBL is treated with a completely different chemotherapy regimen compared to PCM. PBL carries a guarded prognostic profile among DLBCLs with high relapse rate and poor median survival. We present a case of a 44-year-old HIV-positive man who presented with a large retroperitoneal mass associated with obstructive uropathy, sacral radiculopathy, and inferior vena caval compression. The mass was initially mistaken to be a PCM on histopathology; however, subsequent investigations revealed an extra-oral PBL with plasmacytic differentiation. To our knowledge, this will be the first case of PBL of the retroperitoneum in an HIV- and HCV-positive patient and the second one at this location in the English-language literature. In this report, key differentiating points between PBL versus PCM and newer therapeutic agents such as proteasome inhibitors have been discussed along with related review of literature.
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PMID:Plasmablastic lymphoma of the retroperitoneum in an HIV- and HCV-positive patient: hard to diagnose and harder to treat. 2256 57

Plasmablastic lymphoma shares many morphologic features with plasmablastic plasma cell myeloma. The activation of MYC oncogene in these lymphomas may be an important pathogenetic element associated with Epstein-Barr virus infection. We describe herein an elderly man with a plasmablastic lymphoid neoplasm displaying unique morphologic, cytogenetic and clinical features. This case might offer additional insights to the complex but fascinating topic of hybrid haemato-lymphoid neoplasms such as plasmablastic lymphoma-myeloma. In addition, the patient responded to the treatment with bortezomib. Newer antimyeloma agents such as bortezomib have shown promise in the treatment of these neoplasms and should further be explored for their therapy.
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PMID:Plasmablastic haemato-lymphoid neoplasm with a complex genetic signature of Burkitt lymphoma responding to bortezomib. 2289 91


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