A 68-year-old White man presents to his primary care physician with fatigue, fever, night sweats, abdominal pain, and nausea as well as a 15-lb unintentional weight loss in the past 3 months. On physical exam, the patient is pale and tired looking with abdominal distention (palpation confirms hepatosplenomegaly) as well as generalized lymphadenopathy. His medical history includes previous Epstein-Barr virus infection in his early 20s, hypertension, and elevated cholesterol, which are pharmacologically well controlled. His ECOG performance status is 2. The patient is now retired but spent 40 years working on a large farm with significant exposure to various pesticides. He never smoked.

Laboratory studies reveal a hemoglobin level of 8 g/dL, confirming anemia, as well as elevated AST/ALT, LDH > 500 U/L, and lymphocyte count of 4500/µL. CT confirms hepatosplenomegaly and reveals appendiceal enlargement, bowel wall thickening, multiple polyps (presence of both sessile and polypoid polyps) in the gastrointestinal tract, and lymphadenopathy.