Plasmablastic lymphoma(PBL) shows a low incidence and poor prognosis, moreover, there is no standard treatment regimen for PBL. The treatment effect and value of CHOP regimen and radiotherapy are limited. Some studies showed that intensive chemotherapy alone or its combination with proteasome inhibitors or immune regulator can improve the overall survival of patients with PBL, which can be used as the first-line therapy for PBL patients. CAR-T and immunocheckpoint inhibitors showed treatment effect for the patients with refractory and relapsed plasmablastic lymphoma. The clinical value of potential targets in treating tumour worth to be studied further.
Humans ; Plasmablastic Lymphoma

OBJECTIVETo report a case of primary cardiac plasmablastic lymphoma to investigate its clinical feature, diagnosis, therapy and prognosis.

METHODSA case of primary cardiac plasmablastic lymphoma was studied. The imaging examination, conventional histopathological and immunohistochemical staining of this case were detected. The clinical feature, pathogenesis, diagnosis, therapy and prognosis of primary cardiac plasmablastic lymphoma were further investigated through literatures review.

RESULTSThe tumor was located in the right atrium. Microscopic examination showed diffuse proliferation of large lymphoid cells. The neoplastic cells were positive for CD38 and CD79a. The patient was treated with chemotherapy combined with autologous stem cell transplantation.

CONCLUSIONPrimary cardiac plasmablastic lymphoma was extremely rare. Its pathogenesis remained to be unclear. With non- specific clinical manifestations, the diagnosis was mainly confirmed by histopathological and immunohistochemical staining method. Without standard treatment, more patients were treated with chemithreapy regimens similar to the treatment used in aggressine lymphoma. Patients usually had a poor prognosis.

Heart Neoplasms ; diagnosis ; therapy ; Hematopoietic Stem Cell Transplantation ; Humans ; Plasmablastic Lymphoma ; diagnosis ; therapy ; Prognosis

OBJECTIVETo study the clinical, pathologic, immunophenotype, molecular characteristics and prognosis of HIV-negative plasmablastic lymphoma (PBL).

METHODSTwelve cases of HIV-negative PBLs diagnosed between 2005 and 2014 in Guangdong General Hospital were identified according to WHO classification of tumors of haematopoietic and lymphoid tissues (2008). The clinicopathologic features and outcome were analyzed and the relevant literatures were reviewed.

RESULTSThe patients were predominantly male (11/12) with a median age of 55.5 years. The tumor cells showed the characteristic combination of immunoblastic/plasmablastic morphology, plasma cell phenotype and high proliferation, no expression of mature B cell markers. 7/10 of the cases were EBER positive. Two cases were positive for C-myc translocation. Four of twelve patients were died.

CONCLUSIONSPBL is a rare, aggressive B-cell lymphoma. HIV-negative PBL has lower rate of oral involvement and EBER expression than HIV-positive patients, the differential diagnosis is very challenging, and the prognosis is worse.

Diagnosis, Differential ; Female ; HIV Seronegativity ; Humans ; Immunophenotyping ; Lymphoma, B-Cell ; diagnosis ; Male ; Middle Aged ; Plasma Cells ; classification ; Plasmablastic Lymphoma ; diagnosis ; pathology ; Prognosis ; Translocation, Genetic

OBJECTIVETo investigate the clinicpathologic features and diagnosis of plasmablastic lymphoma (PBL).

METHODSEleven cases of PBL were collected and followed up, with review of the literature. HIV and EBV status and their relationships with the tumor were specially compared as well.

RESULTSIn the current cohort, 10 patients were serologically HIV negative; the male to female ratio was 8 to 3, and the median age was 57 years. Ten cases showed extranodal involvement and one case was nodal based. At presentation, five patients had mid-facial involvement, including sinonasal area (3 cases) and oral cavity (2 cases). Histologically, six were PBL of oral mucosa type, and five were PBL with plasmacytic differentiation. In all cases, the neoplastic cells expressed CD138 and MUM-1, and were negative for CD20 and CD3ε; the median Ki-67 index was 80%. Five cases were EBER1/2 in situ hybridization positive. IgH or/and Igκ gene rearrangement was detected in all five cases examined.

CONCLUSIONSMost patients were no congenital or acquired immunodeficiency in the retrospective study. Of the died patients, EBER1/2 in situ hybridization were negative and their disease staging were Ⅳ, The neoplastic cells were immunoblastic or plasmablastic, sometimes the plasmacytoid cell can be seen and the neoplastic cell had mature plasma cell phenotype, the pathologic diagnosis of the lymphoma is still controversial now. Differentiate with plasma cell neoplasm is difficult, it is necessary to accumulate more cases for advanced study and observation in the future.

Female ; Gene Rearrangement ; Humans ; In Situ Hybridization ; Male ; Middle Aged ; Multiple Myeloma ; Plasma Cells ; Plasmablastic Lymphoma ; diagnosis ; pathology ; RNA, Viral ; metabolism ; Retrospective Studies