Case Report Plasmablastic haemato-lymphoid neoplasm with a complex genetic signature of Burkitt lymphoma responding to bortezomib Constantin A. Dasanu 1 * , Frank Bauer 2 , Ion Codreanu 3 , Premkumar Padmanabhan 4 and Murtuza Rampurwala 4 1 Department of Hematology–Oncology, Saint Francis Hospital and Medical Center, Hartford, CT, USA 2 Department of Anatomic and Clinical Pathology, Saint Francis Hospital and Medical Center, Hartford, CT, USA 3 Department of Medical Imaging, University of Arizona Medical Center, Tucson, AZ, USA 4 Department of Internal Medicine, University of Connecticut Health Science Center, Farmington, CT, USA *Correspondence to: Constantin A. Dasanu, Department of Hematology–Oncology, Saint Francis Hospital and Medical Center, Gothic Park, 43 Woodland Street, Suite G-80, Hartford, CT 06105 USA. E-mail: c_dasanu@yahoo.com Received 19 June 2012 Revised 28 July 2012 Accepted 2 August 2012 Abstract 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. Copyright © 2012 John Wiley & Sons, Ltd. Keywords: plasmablastic lymphomas (PBL); plasma cell myeloma; immunosuppression; Burkitt lymphoma; bortezomib Several aggressive B-cell lymphomas have an immunologic profile resembling the plasma cell stage of differentiation [1]. These include plasmablastic lymphoma (PBL) that represents a neoplasm of B-cell lineage, sharing many cytomorphologic and immunophenotypic features with plasmablastic plasma cell myeloma [2]. Although earlier thought to occur almost exclusively in the setting of HIV infection, cases of PBL have been reported in HIV-negative patients with decreased immune surveillance, including the elderly [1,3]. Case report An 80-year-old White man presented to his urologist’s office with a sudden onset of right-sided flank pain in July 2011. Medical history consisted of hypertension, conges- tive heart failure, dyslipidaemia and a remote history of prostate cancer, for which he underwent external beam radiation therapy more than 10 years prior. His medications included metoprolol, simvastatin and low-dose aspirin. His lung and cardiovascular examination was normal, and he had no appreciable peripheral adenopathy or hepatospleno- megaly. Laboratory data showed a haemoglobin count of 11.3 g/dL and an international normalized ratio of 1.2. White blood cell count and platelets were normal; however, lymphopaenia of 300/mcl was documented by an automatic differential. Liver and kidney function test- ings were also normal. A computed tomography (CT) scan of the abdomen and pelvis without contrast demonstrated bilateral retroperitoneal ‘fluid’ collections, greater on the right than on the left, opined by the radiologist as ‘most consistent with hematomas’. Of note, the patient had a history of a cardiac arrest during the administration of in- travenous contrast several years ago, and hence an imaging study with contrast was not pursued. As a result, the patient was managed conservatively for a presumed spontaneous retroperitoneal haemorrhage with serial blood counts. Over the next several weeks, he developed increasing flank pain coupled with subjective fevers, nausea, vomiting, decreased appetite, fatigue and a 10-lb weight loss. On examination, he now had abdominal distention and bilateral 2–3+ lower extremity oedema, greater on the right side. Repeat laboratory tests were signi ficant for a haemoglobin count of 9.2 g/dL, serum creatinine level of 1.1 mg/dL and a lactate dehydrogenase level of 1155 U/L (normal, 140–626 U/L). Serum protein electrophoresis revealed two monoclonal protein spikes, M1 = 1.07 g/dL and M2 = 0.22 g/dL. Immunofixation demonstrated mono- clonal IgM type kappa and free light chain kappa, respec- tively. IgM was increased at 1729 mg/dL (normal, 56–352 mg/dL); IgA and IgG were reciprocally depressed. Beta-2-microglobulin was elevated at 5.05 mg/L (normal, 1.2–2.8 mg/L). CD4 + T cells were 180/mm 3 ; CD8 + T cells were 75/mm 3 . Whereas HIV testing came back negative, Epstein–Barr virus (EBV) serology established evidence of a past infection. After premedication, he underwent a CT scan of the abdomen and pelvis with contrast that revealed enhancement of the retroperitoneal soft tissue abnormality with compression and displacement of the right renal vein, Hematological Oncology Hematol Oncol (2012) Published online in Wiley Online Library (wileyonlinelibrary.com) DOI: 10.1002/hon.2024 Copyright © 2012 John Wiley & Sons, Ltd.