Plasmablastic Lymphoma of Small Intestine: A Rare Case Report with Review of Literature

Abstract

Plasmablastic lymphoma (PBL) is a rare aggressive neoplasm characterized by diffuse proliferation of large neoplastic cells with plasma cell immunophenotype. Cell of origin of PBL is believed to be a postgerminal center B-lymphocyte or plasmablast. The malignant cells in PBL usually do not express CD20 (B cell marker) but do express markers of plasmacytic differentiation, such as CD38, CD138, or MUM1/IRF4, akin to plasma cell myeloma (PCM). PBL though originally described in the oral cavity, has now been found to occur in extraoral locations as well. Small intestine as a site of PBL has been described very rarely. PBL remains a diagnostic challenge given its overlapping morphologic and immunophenotypic features with other high grade lymphomas and PCM. We report a rare case of PBL of small intestine in a 48 years old HIV infected male patient. To the best of our knowledge this represents sixth case in the literature described in this location. An unusual rare pattern of CD138 positivity by IHC is also reported along with extensive review of literature of PBL in extraoral locations. /L. CECT scan of abdomen revealed circumferential diffuse wall thickening of jejunal loop and enlarged mesenteric lymph nodes (Fig. 1). After preoperative evaluation he underwent explorative laparotomy, resection of small bowel with end to end anastomosis and omentectomy. Case ReportScreening tests for HIV I & II done by enhanced chemiluminescence method were reactive with test values being 85.1 (C1.0 reactive, gray zone 0.9-0.99,\0.9 non reactive). Absolute CD3, CD4 and CD8 counts and percentages were assessed by flow cytometry (FCM) technique as showed in Table 1. Serum LDH and Uric acid levels were 702U/L (normal range 208-320U/L) and 4.7 mg/dl (normal range 3.5-7.2 mg/dL). Human immunodeficiency virus (HIV)-viral load as estimated by RT-PCR was 1,536,000 copies/ml (lowest limit of detection 40 copies/mL). Patient underwent explorative laparotomy, resection of small bowel, end to end anastomosis and omentectomy.We received a segment of small intestine measuring 55 cm in length. On cutting open, an ulceroproliferative lesion measuring 7.5 9 3 9 1.5 cm was identified. The lesion was involving the intestine circumferentially. Corresponding serosal surface was irregular. The fat along mesenteric border appears involved. Omentum measured 35 9 5 9 2 cm and showed firm grey white areas.Microscopic examination showed a tumor composed of large to intermediate sized lymphoid cells in sheets infiltrating the full thickness of bowel wall extending into adjacent fat with serosal involvement. The cells were large with round nuclei with small to prominent nucleoli and scanty cytoplasm ( Fig. 2A). Some cells showed plasmacytoid morphology.

Plasmablastic Lymphoma of the Small Intestine in an HIV- and EBV-negative Patient

“…Of the 19 cases, 15 were males, 4 were females, and the median age was 50 years (range 17-85 years). Three males and two females ranging in age from 38 to 48 years (median 40 years) were positive for both HIV and EBV ( 3 , 6 - 9 ). Among these 5 cases, the underlying diseases were HIV and EBV infection in 4 cases and unknown in 1 case.…”

Gastrointestinal Tract Plasmablastic Lymphoma in HIV-Infected Adults: A Histopathological Review

“…The majority of the gastrointestinal tract PBLs have been described in the stomach, small intestine, and colon as case reports and small case series. 18 -26 Herein, we report on 17 patients with gastrointestinal tract PBLs focusing on the morphological and immunohistochemical features.…”

“…8 The findings of the present study show comparable features to those reported in the case studies and series on gastrointestinal tract PBLs. 18 -26…”

“…Currently, studies available include few isolated cases and 2 case series of 4 cases each. 18 -26 Therefore, the current study aimed to comprehensively describe histopathological, immunophenotypical, and molecular features of gastrointestinal tract PBLs to improve on diagnostic accuracy and avoid inappropriate treatment.…”