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

Abstract

Extramedullary hematopoiesis occurs in the setting of hematologic disorders or malignancies when the activity of the bone marrow is insufficient to generate blood cells. We report a unique case of adrenal extramedullary hematopoiesis diagnosed in a 16 year old female with a history of anti-Diego antibody and congenital dyserythropoietic anemia. She presented with an enlarging adrenal mass and underwent surgical resection. Pathology revealed extramedullary hematopoiesis. On literature review, we identified only two prior existing cases of adrenal extramedullary hematopoiesis in pediatric patients, with no prior case reports of adrenal extramedullary hematopoiesis occurring in patients with anti-Diego antibody or in those with congenital dyserythropoietic anemia.

Keywords: Adrenal extramedullary hematopoiesis; Congenital dyserythropoeitic anemia; anti-Diego antibody.

Fig. 1.

Imaging and pathology of adrenal mass. (A) Computerized tomography demonstrated a 5.2 × 4.3 × 4.8 cm mass obliterating the right adrenal gland (white arrow).(B) Photo of gross cross-section of right adrenal mass demonstrating adrenal tissue (white arrow) crowded by extramedullary hematopoiesis (C) Photomicrograph of hematoxylin and eosin staining showing normal adrenal tissue (white arrow) surrounded by hematopoietic precursors (10× magnification, scale bar represents 0.3mm) (D) Photomicrograph of hematoxylin and eosin staining demonstrating higher power view (40× magnification, scale bar represents 0.1mm) of hematopoietic precursors including megakaryocytes (black arrow), myeloid precursors (gray arrow), and erythroid precursors (white arrow).