Unusual presentation of duodenal plasmablastic lymphoma in an immunocompetent patient: A case report and literature review

Abstract

Plasmablastic lymphoma (PBL) is a rare and recently described entity of large B-cell lymphoma. It predominantly occurs in the oral cavity of human immunodeficiency virus (HIV)-positive patients and exhibits a highly aggressive clinical behavior without effective treatment. Recently, sporadic cases describing PBL in extraoral locations of HIV-negative patients have been reported; frequently in patients with underlying immunosuppressive states. To develop the understanding of PBL, the current study reports the unusual presentation of duodenal PBL and reviews the pathogenesis, immunohistochemical features, clinical and differential diagnoses, as well as the treatment of PBL as described in previous studies. The case of a 75-year-old female with duodenal PBL without definite immunosuppression is presented in the current report. The tumor was composed of large B-cell-like cells, and was positive for cluster of differentiation 138 and melanoma ubiquitous mutated-1, with ~80% of the tumor cells positive for Ki-67. The features of the tumor were as follows: Extraoral location, HIV-negative, immunoglobulin M λ-type M protein expression, light chain restriction (monoclonal) and Epstein-Barr virus-encoded small RNA-negative, which are considered to be unusual for PBL. These unusual features complicate the differentiation of PBL from other plasma cell diseases. To the best of our knowledge, this is the first study to report a case of duodenal PBL in an immunocompetent patient. To date, the standard treatment of PBL remains elusive, however, the most commonly administered chemotherapy treatments are CHOP [intravenous cyclophosphamide (750 mg/m2, day 1), intravenous doxorubicin (50 mg/m2, day 1), intravenous vincristine (1.4 mg/m2, day 1) and prednisone (100 mg, days 1–50)]-like regimens. The patient was administered two cycles of CHOP chemotherapy for 56 days, however, ultimately succumbed as a result of disease progression. Therefore, PBL represents a diagnostic and therapeutic challenge. PBL must be considered in the differential diagnosis of gastrointestinal tumors in daily practice, even in immunocompetent patients. Furthermore, CHOP does not appear to be an optimal treatment regimen and more intensive regimens are required.

Unusual bilateral kidney and duodenal plasmablastic lymphoma presentation in an elderly patient: A case report

“…In the present case, Ki-67 expression in duodenal biopsies was 99%. Moreover, Cao et al ( 15 ) reported a case of HIV- and EBER-negative duodenal PBL with Ki-67 expression of ~80%, wherein chemotherapy regimens such as CHOP included i.v. treatment of cyclophosphamide (750 mg/m 2 ; day 1), doxorubicin hydrochloride (50 mg/m 2 ; day 1), vincristine (1.4 mg/m 2 ; day 1) and oral prednisone (100 mg; days 1-50).…”

Unusual presentation of plasmablastic lymphoma involving ovarian mature cystic teratoma: a case report

“…PBL was originally reported exclusively in the jaw and oral mucosa of male, predominantly HIV-positive, patients [ 5 ]. It may be present in patients who are HIV-negative, but otherwise immune compromised such as patients with solid organ or bone marrow transplantation or those being treated for autoimmune disorders [ 6 ]. Cases of PBL occurring in elderly, immune competent patients have been documented and increasingly described in extra-oral locations [ 1 , 6 , 7 ].…”

“…It may be present in patients who are HIV-negative, but otherwise immune compromised such as patients with solid organ or bone marrow transplantation or those being treated for autoimmune disorders [ 6 ]. Cases of PBL occurring in elderly, immune competent patients have been documented and increasingly described in extra-oral locations [ 1 , 6 , 7 ]. Extra-oral PBL has been reported in various locations; however to the best of our knowledge, PBL was never described within ovarian cystic teratoma.…”

“…HIV infection, immune suppression and EBER positivity favor PBL diagnosis, while the presence of monoclonal paraproteinemia, lytic bone lesions, hypercalcemia and renal dysfunction favors a diagnosis of myeloma. Also high Ki-67 proliferation index and CD56 positivity favors diagnosis of PBL over myeloma [ 6 ].…”

Light chain cast nephropathy caused by plasmablastic lymphoma of the bladder

“…In patients with HIV, PBL most often originates from the oral cavity. In case reports of PBL in non-HIV patients, PBL originates from other sites such as the retroperitoneum, gastrointestinal (GI) tract, and lungs [ 4 , 5 , 6 , 7 ]. PBL involving the urinary tract is extremely unusual, and renal failure in association with PBL is rarely reported [ 8 ].…”