Plasmablastic Lymphoma with Primary Impairment of Bone Marrow in a HIV-Negative Patient: A Literature Review and a Case Report

Abstract

Background. Plasmablastic lymphoma (PBL) is a rare variant of large B-cell lymphoma. This disease is usually associated with HIV infection and is predominantly identified in male patients. Tumor lesion is typically localized in oral cavity. PBL is characterized by aggressivity and low rate of long-term survival. Aim. To report a clinical case of a rare localization of PBL with primary impairment of bone marrow in a 19-year-old HIV-negative patient. Materials & Methods. The diagnosis of the disease turned out to be challenging and was based on the results of a multi-step complex immunohistochemical analysis of a bone marrow core biopsy sample. Results. Intensive block-based mNHL-BFM-90 polychemotherapy combined with bortezomib and daratumumab resulted in remission which allowed to perform consecutive autologous and then allogeneic hematopoietic stem cell transplantations. For the lack of immune control of transplant over the tumor the conducted therapy was disappointingly unsuccessful. The patient died in 11 months after diagnosis because of tumor progression. Conclusion. New approaches are definitely called for in order to explore methods of treating this complex disease. A study of mechanisms underlying PBL pathogenesis can contribute to better understanding of tumor biology and personalized choice of chemotherapy.