A Unique Presentation of Cutaneous T-cell Lymphoma Involving the Gastrointestinal Tract

Abstract

Introduction: Most cases of lymphoma involving the gastrointestinal (GI) tract are of B-cell lineage. T-cell lymphoma affecting the GI tract is infrequent. Cases of lymphoma with GI involvement mainly involve primary lesions and not secondary disease. Herein, is a patient with cutaneous T-cell lymphoma (CTCL) who presented with a bowel perforation and found to have secondary T-cell lymphoma of the small intestine. Case: A 74-year-old Caucasian female with primary CTCL (Figure 1) treated with conventional chemotherapy agents and radiation of large areas of her skin presented with one-month history of periumbilical abdominal pain. Admission labs revealed normocytic anemia, severe thrombocytopenia, elevated transaminases, and hyperbilirubinemia. Computed tomography of the abdomen and pelvis with oral contrast was concerning for perforated viscus in the pelvis, most likely communicating with the cecum with what appeared to be extravasation of oral contrast (Figure 2). Exploratory laparotomy revealed wide-open perforation of distal ileum, matted interloop adhesions of small bowel, with distal ileal obstruction, and fecal peritonitis. She underwent a resection of the distal ileum with reanastomosis. Immunohistochemistry and histopathology from jejunal and distal ileum biopsies were consistent with T-cell lymphoma (Figure 3). The patient had recurrent refractory T-cell lymphoma now with bowel metastasis. Given her poor prognosis, the patient transitioned to hospice. Discussion: GI lymphoma represents up to 10% of primary GI malignancies with B-cell lymphoma occurring more often than T-cell lymphoma. CTCL, a type of non-Hodgkin lymphoma, is diagnosed by the presence of malignant T-cells in chronically inflamed skin. Early presentation is mostly limited to skin patches or plaques, but in one third of patients the disease becomes progressive and spreads to blood, lymph nodes, and visceral organs. The most frequent site of GI involvement is the stomach, followed by the small intestine and ileocecal area.A study that reviewed the incidence of perforation in biopsy-proven GI involvement lymphoma found that 59% of perforations were associated diffuse large B-cell lymphoma. In this case, the patient had secondary T-cell lymphoma, which is infrequent, and was found to have a perforation which is uncommon. Patients with a history of lymphoma who present with new onset abdominal pain should be evaluated for free air in the abdomen to rule out perforation.2568_A Figure 1. Wound on the left lower extremity2568_B Figure 2. CT abdomen and pelvis with oral contrast showing extraluminal extravasation of contrast in pelvic cul-de-sac2568_C Figure 3. T-cell marker (CD3) expression in neoplastic cells from small bowel biopsy (A) and hematoxylin and eosin stain showing lymphocyte predominance from small bowel biopsy (B)