2A81.2/C83.3 Plasmablastic lymphoma

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Aetiology

Plasmablastic lymphoma (PBL) is a rare form of non-Hodgkin lymphoma that is associated with human immunodeficiency virus (HIV) infection. It is an uncommon type of large B-cell lymphoma that is characterized by an aggressive clinical course and poor prognosis.

Diagnosis

Diagnosis of PBL is based on clinical, histologic, immunophenotypic, and molecular features. Histologically, the tumor cells are large, pleomorphic, and have an immunoblastic or plasmablastic appearance. Immunophenotypically, the tumor cells are positive for CD20, CD79a, CD138, and MUM1. Molecular studies have demonstrated that PBL is associated with a translocation of the c-myc gene, which is not seen in other B-cell lymphomas.

Differential diagnosis

The differential diagnosis of PBL includes other large B-cell lymphomas, such as Burkitt lymphoma, diffuse large B-cell lymphoma, and immunoblastic large cell lymphoma.

Treatment

Treatment of PBL is based on the stage of the disease. In early-stage disease, chemotherapy with rituximab is recommended. For advanced-stage disease, chemotherapy plus radiation or a stem cell transplant may be recommended.

Prognosis

The prognosis for patients with PBL is poor, with a median survival of 6-7 months. However, the prognosis is better for patients with early-stage disease.

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