S2854 Peripheral T-Cell Lymphoma, Not Otherwise Specified (PTCL, NOS) Presenting With Gastrointestinal Bleeding in an HIV Patient

Abstract

INTRODUCTION: Peripheral T-Cell lymphomas (PTCL) are a rare type of Non-Hodgkin’s Lymphoma (NHL) with an incidence of < 1 case per 100,000 people in the USA. PTCL, not otherwise specified (NOS) is an EBV-associated malignancy, encompassing a spectrum of disorders that do not fulfill the criteria of other PTCL subtypes. Presentation of PTCL, NOS with gastrointestinal bleeding has only been described in rare case reports. CASE DESCRIPTION/METHODS: A 32-year-old female with congenital HIV, on highly active anti-retroviral therapy (HAART), presented with multiple episodes of hematochezia, diffuse abdominal pain and hypotension. After hemodynamic stabilization with multiple units of packed red blood cells and intravenous fluids, esophagogastroduodenoscopy and colonoscopy were performed revealing no obvious source of bleeding. Subsequently, CT scan and MRI of the abdomen delineated two segments of small bowel wall thickening, luminal narrowing and mesenteric lymphadenopathy. Capsule endoscopy showed scattered patches of erythematous mucosa in the small bowel without active bleeding or stigmata of bleeding. Afterwards, she underwent a small bowel push enteroscopy revealing a large, non-obstructing, infiltrative mass in the mid-jejunum which was successfully biopsied (Figure 1). Biopsy was consistent with PTCL, NOS with a high Ki-67 proliferation index (80-90%) and positive Epstein-Barr encoding regions-in situ hybridization (EBER-ISH) (Figure 2, 3). Further staging workup showed a normal bone marrow biopsy. PET-CT scan demonstrated intense FDG uptake in jejunum and small bowel segments in the left and right lower quadrants but no distant metastases. During the preparation for chemotherapy, she developed acute small bowel perforation. Given the rapid deterioration, definitive treatment was not feasible. After family discussions, she was transferred to inpatient hospice where she passed away. DISCUSSION: Push enteroscopy is an effective tool for the diagnosis of small bowel malignancies like PTCL. The presence of HIV increases the risk of PTCL by 15 times and adversely impacts the prognosis, with a median survival of around 1 year. Around 30% cases of PTCL, NOS are EBV positive and the presence of EBER-ISH portends worse outcomes. Induction with anthracycline-based chemotherapy regimens along with brentuximab vedotin (an antibody-drug conjugate against CD30) for CD30 + PTCL, and early consolidation with autologous stem cell transplant (ASCT) are the cornerstones of PTCL, NOS treatment.Figure 1.: Small bowel push enteroscopy: Panel (A) and (B) show a prominent mass lesion in the mid-jejunum. Panel (C) and (D) show the same area after biopsy and tattooing.Figure 2.: Small bowel mucosa with large pleomorphic cells infiltrating the lamina propria. Surface ulceration and extensive areas of punctate necrosis are also present. Figure (A) is X10 and Figure (B) is X25 magnification.Figure 3.: The large pleomorphic cells are positive for (A) CD3 (B) CD7 and (C) CD8 immunostains as well as (D) EBER-ISH (X25).