Seventy-four-year-old woman with myelodysplastic syndrome and splenomegaly.

We present a 74-year-old woman with a recent diagnosis of myelodysplastic syndrome who presented with left upper quadrant abdominal pain and fatigue with significant splenomegaly, anemia, and thrombocytopenia. She underwent splenectomy and bone marrow biopsy. Pathology of both spleen and bone marrow revealed an unusual diagnosis. A review of the differential diagnosis, laboratory tests, nature of the underlying disease, and treatment are provided.