Asymptomatic spherocytosis presenting with spinal cord compression: case report

Extramedullary hematopoiesis (EMH) is a compensatory mechanism occurring in patients with chronic anemia, which occurs most frequently with thalassemia. The authors report the case of a 57-year-old man, with no history of clinical or hematological disease, presenting with spinal cord compression. Magnetic resonance (MR) imaging demonstrated a homogeneous posterior epidural mass extending from T-3 to T-6. Following decompressive surgery, the patient's symptoms improved. Histological analysis showed features consistent with a diagnosis of EMH. Subsequent workup was remarkable for an asymptomatic spherocytosis without anemia. There was no family history of anemia. An EMH-related presentation of mild spherocytosis has been described in the literature, but its epidural location led to spinal cord compression. The MR imaging features were suggestive of EMH, but in the presence of spinal cord compression and in the absence of a history of chronic anemia, the authors did not believe that nonsurgical management would have been reasonable.