Case Series of Paraspinal Extramedullary Hematopoiesis in Transfusion-Dependent Thalassemia Treated with Luspatercept

Introduction: Extramedullary hematopoietic (EMH) pseudo-tumors are commonly seen in patients with non-transfusion dependent thalassemia but rare in transfusion-dependent beta thalassemia (TDT). Paraspinal EMH is more prevalent in older patients with severe ineffective erythropoiesis and low fetal hemoglobin levels. Luspatercept, an inhibitor of the TGF-beta pathway, has been shown to reduce transfusion requirements and improving iron overload in TDT. Emerging data report risk of EMH in patients receiving luspatercept, resulting in updated safety labeling, yet there is scarce data on the optimal management of EMH. In this case series, we present four cases of paraspinal EMH in TDT patients in their twenties treated with Luspatercept, and report management approaches and treatment response.