A 31-year-old Ashkenazi Jewish male presented to his primary care physician due to early satiety, nausea, abdominal discomfort, and occasional vomiting. Laboratory tests were within normal limits, and abdominal ultrasound demonstrated splenomegaly. He was referred to our hospital after a follow-up total body computed tomography (CT) scan that demonstrated a massively enlarged spleen, measuring 28 cm in its greatest dimension (Fig. 1A), with numerous hypodense lesions measuring up to 6 cm in diameter.
