Rare Presentation of Right Adrenal Mass: Extramedullary Haematopoiesis in a Patient with Thalassaemia Intermedia

Abstract

Extramedullary hematopoiesis (EMH) is a rare cause of adrenal mass. We present a 44-year-old woman who has thalassaemia intermedia, referred to Endocrinology clinic for huge adrenal mass. Along with a paraspinal lesion discovered in this patient, the leading diagnosis was EMH. The patient was treated with hypertransfusion and hydroxyurea, which led to a reduction in the size of the right adrenal mass and paraspinal mass.This case highlights the challenges in managing this rare condition. Although EMH is a rare cause of adrenal mass, the diagnosis must be considered in any patient with a history of a congenital hemolytic disorder, to avoid unnecessary surgical procedures.

Adrenal extramedullary hematopoiesis in the setting of anti-Diego antibody and congenital dyserythropoietic anemia

“…The Diego blood group system was first described in 1955 when it was found to cause fatal hemolytic disease of the newborn [ 5 ]. There are a total of 22 antigens within the system and their impacts range from clinical insignificance to hemolytic transfusion reactions, as seen in our patient, or to hemolytic disease of the newborn [ 6 ] In adult case reports, adrenal extramedullary hematopoiesis has been reported in patients with thalassemia [ 7 – 16 ], sickle cell disease [ 17 ], primary myelofibrosis [ 18 ], and myeloid metaplasia [ 19 ] The discovery of a patient with Diego blood antigens in this setting is a unique finding within the existing literature.…”