Clinical Vignettes/Case Reports - Small Intestine/Unclassified.

Abstract

Peripheral T-Cell Lymphoproliferative Disorder (PTCL) is a rare group of neoplasms and represents less than 5% of all Non-Hodgkin Lymphomas. They occur more commonly in adults with a median age of 60 years but rarely reported in the oldest old (more than 85 years). Usually it's described as diffuse pattern of infiltration by polymorphic atypical neoplastic T-cells. This entity has vague clinical presentation including fever, night sweats and weight loss, frequently difficult to diagnose. This is a functionally independent 86 year old Jamaican Female with history of esophageal reflux known patient of our Gastroenterology service, who came for evaluation of early morning satiety, nausea and significant progressive weight loss in a period of 6 months. Her physical examination was significant only for mild epigastric discomfort. Family and Social history, surgical procedures, laboratory findings, chest radiography and abdominal ultrasound were unremarkable. In the previous year, CT-Scans and MRI of the abdomen because of reflux symptoms were normal. The current imaging found a soft tissue mass 3.9x7 cm in the mid abdomen rising from the small bowel, abnormal loops with thickened wall, indistinct margins and extensive mesenteric and retroperitoneal adenopathy (Figure I). She underwent push enteroscopy exposing a partially circumferential frond-like/villous jejunal friable mass with no bleeding at 50cm beyond the ligament of Treitz, extending 5 cm with non-obstructing characteristics (Figure II). The histology revealed villous blunting, ulceration and mixed inflammatory erosion in the lamina propia and a heavy atypical lymphoid infiltration deeper in the submucosa. The immunohistochemical analysis confirmed positive CD-3 with CD5 & CD4: T-Cell phenotype with negative CD30 and ALK-1. In accordance with the Ann Arbor system, this is considered stage IIE lymphoproliferative disorder. Further evaluation to obtain a final diagnosis was attempted but declined by the patient and the inability to undergo surgical resection considering her very poor pulmonary status. On follow up, no new symptoms were reported. Peripheral T-Cell Lymphoproliferative disorder presenting in the G.I. system is rare and has not been reported in the oldest old (more than 85 years old). Its differential diagnosis includes HTLV-1 or Enteropathy Associated T Cell Lymphoma. The value of this case is its unusual presentation in such uncommon pathology for the geriatric population.Figure 1Figure 2